STRUCTURE OF SKIN
Skin is the largest organ of the body. It is a very complex structure composed of three layers viz., epidermis, dermis, and subcutaneous tissue. It is also composed of various appendages. The outermost layer or the epidermis is further composed of four layers, stratum corneum, stratum granulosum, stratum spinosum, and stratum basale. There are melanocytes situated at the basal cell layer and Langerhan cells throughout the epidermis.
The dermis is predominantly composed of collagen and elastic fibers. There are cell types including fibroblasts, macrophages and mast cells in the dermis. Various structures situated in the dermis are composite structure of hair follicle with sebaceous gland known as pilosebaceous apparatus, and eccrine or sweat glands. Beneath the dermis, there is a layer of fat known as subcutaneous tissue. In the dermis, there are dermal capillaries and venules which comprise dermal vasculature. There are also lymphatics and nerve endings situated in the dermis.
FUNCTIONS OF SKIN
The outermost layer or stratum corneum performs the barrier function and maintains the hydration of skin. It also prevents the penetration of irritants, toxins, and organisms through the epidermis into the dermal capillaries from the environment. The rest of the epidermis acts as protective layer. Melanocytes of the epidermis confer color of the skin by transferring melanin to the basal keratinocytes. It also protects the skin from deleterious effect of ultraviolet light. Langerhan cells present antigen to the immunological unit of the skin. Hence, it is considered as the first line of immunologic defense.
The collagen and elastic fibers act as a tough, leathery, mechanical barrier against cuts, bites, abrasions, and bruises. Its collagenous matrix provides structural support for various cutaneous appendages. Hair grows from follicles placed in the deep dermis and imparts beauty to the individual. It also protects the scalp from the sunlight and various environmental allergens. Sebaceous glands produce oily secretions, lubricate the skin and contribute to the protective function of epidermal barrier. Eccrine glands produce sweat which secrete on the skin surface through eccrine ducts. Thus, it acts as an important organ responsible for thermoregulation. Cutaneous capillaries, venules, and lymphatics act as perfusion unit of skin. The skin also contains specialized receptors for heat, pain, touch, and pressure. Sensory inputs from these structures help to protect the skin surface against environmental trauma and noxious agents. The subcutaneous fat acts as a protective cushion for the skin and stores energy and provides insulation to the body. To summarize, various functions of the skin are as follows:
- Protection: It protects the body from physical, chemical, and biological injuries.
- Perception: It perceives various sensations such as pain, touch, temperature, and vibration.
- Temperature regulation: Eccrine sweat glands and dermal vasculature play important role in thermoregulation.
- Barrier function: Skin acts as a permeability barrier that regulates the diffusion of substances particularly water and electrolytes, etc.
- Secretory function: Synthesis of vitamin D3 is an important secretory function with the help of sunlight.
- Storage function: The dermis and subcutaneous fat act as a storage center for energy and other compounds.
- Excretory function: Some of the harmful substances are excreted through the skin.
- Immunological function: Recognition of antigens and elicitation of immunological response is done by skin.
- Cosmetic function: Color and texture of skin along with the hair and nail play an important role in esthetic appeal of an individual.
SKIN TYPES AND SKIN COLOR
Skin color is of two types: Constitutive skin color and facultative skin color.
“Real knowledge is to know the extent of one's ignorance.” –Confucius
Constitutive skin color is the basic skin color of an individual and is genetically determined. Facultative skin color is the skin color that results from ultraviolet ray (UVR) 2exposure. It is basically tanning of the skin. Depending on the response of the skin to UVR, various skin phototypes (SPTs) have been described and different races with different SPTs possess different types of skin color. Table 1 highlights color of skin and response to UVR in different SPTs.
Three types of melanin in humans have been demonstrated—eumelanin (brown-black melanin), pheomelanin (yellow-red melanin), and neuromelanin (black). While in most of the melanocytes there is a minimal admixture of pheomelanin and eumelanin, in red hairs exclusively pheomelanin is present. Neuromelanin is present in nerve cells. Neuromelanin is formed by an enzymatic pathway different from that of eumelanin or pheomelanin synthesis. Hence, in oculocutaneous albinism (OCA) there is presence of pigment in substantia nigra.
BASIC MORPHOLOGY OF SKIN LESIONS
Various morphologies of the lesions have been divided in to three types—primary lesions, secondary lesions, and special lesions.
Primary Lesions
Different types of primary lesions are:
- Macule: It is only the change of color of skin, texture remaining unaltered. A macule can be hyperpigmented, hypopigmented or erythematous.
- Papule: It is a circumscribed solid lesion measuring <1 cm in diameter. A papule can be erythematous, skin-colored or of any other color. The surface may be flat, verrucous, or umbilicated.
- Plaque: It is a solid elevation >1 cm in diameter. There may be associated follicular plugging, telangiectasia, atrophy, etc.
- Nodule: It is a deep-seated solid three dimensional lesion which may be hard, firm, soft, fleshy, tender or nontender, fixed or mobile. The surface of the nodule can be smooth, keratotic, ulcerated or fungating.
- Pustule: It is a circumscribed elevated lesion containing pus. Sometimes it may be sterile.
- Vesicle and bullae: A vesicle is a circumscribed raised lesion containing fluid and <1 cm in diameter. Bulla is a circumscribed raised lesion containing fluid, >1 cm diameter. Vesicle or bullae may be tense or flaccid and on rupture leave behind raw area.
- Wheal: It is a transient or evanescent elevated plaque-like lesion with erythema, edema, and central pallor.
- Cyst: It is a sac that contains liquid or semisolid material.
- Abscess: It is a collection of pus either in the dermis or subcutaneous plane or both.
Secondary Lesions
These are lesions which are basically modified primary lesions induced by scratching, rubbing or secondary bacterial infections. Various secondary lesions are:
- Scales are formed as a result of increased or abnormal keratinization. They may be fine powder-like or large silvery white or even fish like.
- Crust is a collection of dried exudates, dead tissues, and microorganisms. Crust may be brown/brownish-yellow, may be adherent or friable.
- Excoriation or scratch marks are predominantly seen in pruritic disorders.
- Erosion is a superficial ulceration covered with serous exudates and heals without scarring.
- Ulcer is the result of breach in the continuity of skin with loss of epidermis and a part of dermis. Ulcer always heals with scarring.
- Lichenification is thickening, increased criss-cross markings, and mild hyperpigmentation. It is the end result of chronic eczema.
- Atrophy is thinning of skin which appears shiny wrinkled with prominent blood vessels underneath.
“In the practice of tolerance, one's enemy is the best teacher.” –The Dalai Lama
Special Lesions
Some of the lesions are pathognomonic of certain diseases as follows:
- Burrows are tunnel-like serpiginous lesions seen in the superficial part of the skin.
- Comedones are the result of plugging of the pilosebaceous duct by internal secretion. Comedones may be open (black) or closed (white).
- Milia are small superficial cysts 1–2 mm in diameter, commonly seen on the face.
- Telangiectasia means distinctly visible dilated superficial blood vessels.
Characteristic configuration of lesions may suggest a diagnosis. Some classical examples are:
- Linear: Lesions are present in a line. Examples are epidermal nevi, lichen striatus, warts, psoriasis, incontinentia pigmenti, etc.
- Dermatomal: Lesions are present in a dermatome. Examples are herpes zoster, vitiligo, nevus depigmentosus, and PWS.
- Serpiginous: Lesions follow a serpiginous track. Examples are cutaneous larva migrans, and elastosis perforans serpiginosa.
- Annular: Lesions present with central clearing with peripheral border. Examples are tinea, granuloma annulare, erythema annulare centrifugum, and erythema marginatum.
- Herpetiform: Grouped lesions or clustered lesions. Examples are herpes simplex infection, herpes zoster, and dermatitis herpetiformis.
- Reticulated: Means net-like. Examples are cutis marmorata, livedo reticularis, and erythema ab igne.
- Filiform: Means thread-like. Examples are filiform warts, dermatosis papulosa nigra, and skin tags/acrochordons.
- Geographic: Examples are geographic tongue, psoriasis, and erythema annulare centrifugum.
NEONATAL DERMATOSES AND CARE OF NEWBORN SKIN
Most of the newborn disorders are transient in nature requiring only symptomatic treatment. They can be acquired, congenital or developmental disorders.
Mongolian Spot (Figs. 1A to D)
It is the most common type of congenital dermal melanocytosis noted in 90% of Asians. It presents as macular blue-gray pigmentation at birth most commonly on the lumbosacral area in normal infants of darker-skinned races. They are rounded or oval in shape, in variable sizes and usually single or multiple. Less common sites include buttocks, flanks, and shoulders. The pigmentation develops in fetal life, increases in depth for a period after birth and then diminishes. It usually disappears in the first decade of life.
Natural History
It is considered as one of the physiological skin changes of the newborn. When Mongolian spots are associated with bilateral Nevus of Ota, they take much longer time to disappear spontaneously. Persistent and giant Mongolian spots are seen in Niemann-Pick disease, type 1 gangliosidosis, Hurler, and Hunter disease.
Management
Explanation of the condition and its natural history to the parents helps to reassure them.
The sebaceous glands of neonates produce a considerable amount of sebum in first few weeks of life influenced by maternal androgen. There is increased production of dehydroepiandrosterone (DHEA) by fetal adrenal glands. Neonatal acne mostly affects male infants and is seen at birth or within first week of life. Clinically presents as erythematous papules, pustules, and occasionally with comedones distributed on face, more over the cheeks and forehead. Pustular eruption in neonatal acne has been termed as neonatal cephalic pustulosis secondary to colonization of Malassezia species. Child should be evaluated for underlying virilizing tumors or adrenal cortical hyperplasia if there are other signs of hyperandrogenism.
Figs. 1A to D: (A) Bluish-black hyperpigmented patch of Mongolian spot over back; (B) Mongolian spots over back in a newborn; (C) Mongolian spots over back; (D) Extensive Mongolian spots.
“Sharing knowledge is the most fundamental act of friendship. Because it is a way you can give something without losing something.” –Richard Stallman
The lesions are self-limiting and tend to subside by 2–3 weeks and require no treatment. It can persist for 1 year. Benzoyl peroxide 2.5% or 1% salicylic acid has been tried.
Milia are one of the most common transient findings in the cutaneous survey in neonates seen in 40–50% of healthy newborns. These consist of 2–3 mm white or yellow papules on the nose, chin, cheeks, and forehead. Milia are epidermal cysts derived from the pilosebaceous follicle of vellus hair and contain concentric layers of keratin. Widespread and persistent milia can be associated with epidermolysis bullosa, type 1 oro-facial-digital syndrome, and pachyonychia congenita.
Natural History
They are normally spontaneously extruded in a few weeks.
Toxic Erythema of the Newborn (Erythema Toxicum Neonatorum) (Figs. 7A to D)
The cause of toxic erythema of the newborn is unknown and is seen in 48–72% of full-term newborn. In majority, the onset is during the first 48 hours after birth or up to 3 weeks. The eruption can appear as a blotchy macular erythema initially and later develop multiple erythematous papules, their number varying from one to several hundred, most profuse on the anterior trunk, rapidly spreading to involve the face, chest, and extremities. They are often described as “flea bitten appearance” or papules surrounded by a sea of erythema. In severe cases, urticarial papules develop which in 10% cases are surmounted by pustules measuring 2–4 mm in diameter.
“You don't need to see the whole staircase, just take the first step.” –Martin Luther King Jr
Differential Diagnosis
These lesions have to be distinguished from milia, miliaria, transient neonatal pustular melanosis, infantile 5acropustulosis, incontinentia pigmenti, herpes simplex virus infection, varicella, and impetigo.
Diagnosis
Toxic erythema can be diagnosed by microscopic examination of a smear of pustular contents stained with Giemsa and in doubtful cases, cultures to rule out infective etiology can be done. The smear in case of toxic erythema reveals cluster of eosinophils. This is in sharp contrast to bacterial infections where cluster of neutrophils are seen.
Treatment
As the condition is benign, self-limiting, and asymptomatic no treatment is required. It resolves spontaneously by 10−14 days.
Intertrigo (Figs. 8A to F)
Intertrigo is a term applied to an inflammatory dermatosis that is more or less confined to the major body folds and provoked by moisture and constant friction between opposing skin surfaces. Obesity, poor hygiene, overwarm clothing, hot and humid climatic conditions are the predisposing factors. Relative obesity of well-nourished infants accounts for the unusually high incidence during the early months of life. Intertrigo is likely to become colonized and secondarily infected by both bacteria and yeast, particularly Candida albicans. Clinically, it manifests as symmetrical areas of sharply marginated erythema confined to areas of skin apposition. In infants, the folds of the neck, the axillae, the genitocrural flexures, and the intergluteal cleft are the predilection sites. Miliarial lesions are readily apparent within the affected area. When C. albicans infection supervenes, erythema takes on a deep red hue along with maceration, oozing, and satellite pustules. Rarely it may become superinfected with group A streptococci and Corynebacterium diphtheriae.
Management
Management of intertrigo is mainly removal of predisposing factors, cool environment, loose cotton clothes, and topical antibacterial and antifungals. If bacterial superadded infection is evident, a topical antibacterial-like mupirocin may be useful. Topical antifungals such as clotrimazole and miconazole are effective for candidal intertrigo.
“The invariable mark of wisdom is to see the miracles in the common.” –Ralph Waldo Emerson
Miliaria (Figs. 9A to E)
Miliaria occurs when the flow of eccrine sweat is impeded by obstruction of the intraepidermal portion of the sweat duct. 6Miliaria crystallina appears to reflect obstruction of the sweat duct within the stratum corneum. Miliaria rubra occurs when there is sweat duct obstruction deeper in the epidermis.
Figs. 7A to D: (A) Toxic erythema of newborn; (B) Close-up view of lesions; (C) Blotchy macular erythema and satellite pustules of toxic erythema of newborn; (D) Erythema toxicum neonatorum, note bright red erythema and pustular lesions.
Miliaria crystallina presents as crops of clear thin-walled, superficial vesicles, 1−2 mm in diameter without associated erythema resembling water droplets on the scalp, forehead, neck, and upper trunk. They rupture within 24 hours followed by branny desquamation.
Miliaria rubra, seen most commonly in the neonatal period, comprises of grouped to discrete erythematous nonfollicular, papules, and papulovesicles, measuring about 1–4 mm in diameter on a background of macular erythema. Staphylococcal secondary infection of miliaria may lead to sweat gland abscesses. The symmetrical crops of miliaria rubra occur most often in flexural areas, especially around the neck, in the groins, and the axillae. The face, scalp, and upper trunk are frequently affected. These lesions subside in 2–3 days but recurrences are common.
Differential Diagnosis
Differential diagnosis includes neonatal acne, candidiasis, staphylococcal, and herpes simplex infections. Miliaria crystallina is distinguishable from viral infections of the skin by the lack of background erythema and by the absence of inflammatory cells or giant keratinocytes on cytological examination of the vesicles. Miliaria rubra can be distinguished from toxic erythema by its flexural predominance, frequent presence of vesicular lesions and by the tendency to recur.
Management
Avoidance of excessive heat and humidity is the most important aspect of management. Cool baths, light clothing, and installation of air conditioner in the room are helpful. Topical soothing agents such as calamine lotion help in most of the cases. Systemic antibiotics can be given for secondary infection.
“The true sign of intelligence is not knowledge but imagination.” –Albert Einstein
Staphylococcal scalded skin syndrome (SSSS) is caused by an epidermolytic toxin elaborated by certain strains of Staphylococcus aureus, commonly phage group II. The condition is most commonly seen in the first 5 years of life.
Figs. 8A to F: Intertrigo. (A) Intertrigo of inguinal folds; (B) Note erythema over inguinal and perianal area; (C) Note postinflammatory hypopigmentation surrounding erythema; (D) Intertrigo, severe perianal involvement; (E) Severe intertrigo; (F) Intertrigo seen in the neck fold.
“Because the people who are crazy enough to think they can change the world are the ones who do.” –Steve Jobs
It is particularly common during the neonatal period occurring in association with purulent conjunctivitis 8or an upper respiratory tract infection. Sites of predilection are the central part of the face (periorificial), the axillae, and groins. The orange-red, scarlatiniform eruption spreads rapidly. Tenderness of the skin is an early and striking feature. The eruption gradually becomes extensive and turns to a confluent deep erythema and edema in the next 24–48 hours. The surface becomes wrinkled before starting to separate out leaving raw red erosions. Nikolsky's sign is positive. The child is pyrexial and distressed. Radial crusting and fissuring around the eyes, angles of mouth occurs. The only differential diagnosis which poses a problem is toxic epidermal necrolysis, which is however, relatively rare in young children and is characterized by marked mucosal involvement.
Figs. 9A to E: (A) Miliaria rubra and pustulosa in a newborn; (B) Exfoliating miliaria rubra; (C) Miliaria crystalline in newborn; (D) Miliaria pustulosa; (E) Subsiding miliaria, note fine branny scales.
“When one door of happiness closes, another opens; but often we look so long at the closed door that we do not see the other one that has been opened for us.” –Helen Keller
The diagnosis of SSSS can be established by isolation of coagulase positive Staphylococcus aureus from the pyogenic skin lesions or from nostril and areas around eyes.
Figs. 10A to C: (A) Staphylococcal scalded skin syndrome (SSSS), early stage; (B) Impending SSSS, note extensive impetigo bullosa indicating impending SSSS; (C) Full blown SSSS.
Prognosis
Prognosis is good with early diagnosis and appropriate treatment. The condition has a mortality of approximately 5% and fatalities occur mostly in debilitated infants.
Management
Treatment should be started promptly with a penicillinase resistant antistaphylococcal antibiotic such as cloxacillin or a combination of amoxicillin and clavulanic acid or cefadroxil orally. Parenteral antibiotics may be required in cases of extensive skin lesions or severely ill children. Local cleaning of the skin with distilled water or normal saline soaked cotton is sufficient. Antiseptic cleaning is better avoided, so also application of prolonged topical antibiotic for fear of emergence of resistant strains of S. aureus. Parenteral vancomycin is preferred for methicillin-resistant S. aureus infection.
“It is impossible to live without failing at something, unless you live so cautiously that you might as well not have lived at all—in which case you fail by default.” –JK Rowling
10Sucking Blister (Fig. 12)
Sucking blisters are seen in newborn at birth due to vigorous sucking on the affected part by the fetus in utero. Solitary bullae or erosions of 0.5–2 cm are seen over the dorsal aspect of the fingers, thumbs, wrists, lips, and radial aspect of forearms.
Differential Diagnosis
These lesions need to be differentiated from a host of conditions such as bullous impetigo, epidermolysis bullosa, and neonatal herpes simplex.
Treatment
Resolves rapidly without any treatment and sequelae.
“Believe you can and you're halfway there.” –Theodore Roosevelt
Infection of the neonate by herpes simplex virus ranges from mild to potentially life-threatening condition with high mortality. The majority of these infections result from transmission of HSV-1 and HSV-2 from genital tract secretions during delivery. Transmission can occur during intrauterine, peripartum, and postpartum period. There is higher incidence of neonatal herpes (40–50%) during primary maternal infection than in recurrent infection (4–5%). Over 70% of the infection with neonatal HSV has skin or mucosal lesions. There are three distinct forms of neonatal herpes: Skin-eye-mouth (SEM), disseminated, and only nervous system involvement. Skin manifestations are in the form of multiple grouped vesicles on erythematous patches distributed over the scalp, face, trunk, and extremities. The vesicles have a tendency to localize over mucocutaneous junctions. Onset is between birth and 20 days. Oral lesions in the form of erosions of the tongue, palate, gingiva, and buccal mucosa are also common. Primary neonatal herpes simplex is a devastating life-threatening infection especially in the disseminated form involving multiple organs which must 11be diagnosed and treated with antiviral drugs promptly. Complications include pneumonia, seizures, encephalitis, keratitis, chorioretinitis, coagulopathy, and sepsis.
Diagnosis
Diagnosis can be co-established with the help of Tzanck smear preparation, viral culture, use of monoclonal antibodies, and nucleic acid hybridization techniques.
Management
Intravenous acyclovir is the treatment of choice. It is given at a dose of 60 mg/kg/day in three divided doses for 14–21 days. There is no role of topical acyclovir ointment application over the lesions. Prophylactic ophthalmic topical preparations such as trifluridine or vidarabine are helpful.
Neonatal Varicella (Figs. 16A to D)
Neonatal varicella occurs in the first 28 days of life and is due to infection developed by mother during the last few weeks of pregnancy or first few days postpartum or in case of similar infection acquired by other close contacts after birth. Premature infants are at increased risk for nosocomial acquisition of varicella-zoster virus (VZV) because active transfer of maternal IgG antibodies occurs primarily during the third trimester of pregnancy. Neonatal varicella is uncommon in Indian scenario, due to the presence of antibodies in the mother formed on prior exposure to illness.
Two types of varicella infection can occur in the newborn:
- Severe infection: When the mother has been infected less than 5 days before birth or 2 days after delivery (as varicella-associated antibodies are not transmitted to the newborn) or the onset of infection in the newborn is between 5 and 10 days of birth. Disseminated disease may ensue with poor prognosis and high risk of mortality (30%) due to pneumonia, hepatitis, coagulopathy, meningoencephalitis, and secondary severe sepsis.
- Mild infection: If the disease onset in the mother is 5 days prior to birth (antibodies are transmitted to the child) or in the newborn during the first 4 days of life.
Diagnosis of neonatal varicella can be made clinically from the pattern of rash and maternal history of infection. Definite diagnosis is made by virus isolation or amplification of viral DNA from the skin lesions by PCR. Management of newborns who are exposed to VZV by maternal infection (within 5 days before birth or 2 days after delivery) or contact with affected individuals includes isolation and postexposure prophylaxis with varicella-zoster immune globulin (VZIG). Healthy term neonates who are exposed to VZV postnatally (including infants whose mother's rash developed >48 hours after delivery) generally do not require postexposure prophylaxis. VZIG should be given as soon as possible and within 10 days of exposure at a dose of 125 units (1 vial) IM for neonates weighing >2.1–10 kg and 62.5 units (0.5 vial) IM for children weighing ≤2 kg. Intravenous immunoglobulin (IVIG) or IV acyclovir can be considered if VZIG is unavailable. Maternal treatment includes oral acyclovir within 24 hours of onset. Newborns with severe disseminated VZV infection should be treated with IV acyclovir (30 mg/kg/day in three divided doses) for 10 days.
It presents as a bright red shiny polypoidal growth over the umbilicus at birth and is the result of a partially patent omphalomesenteric duct. The lesions are usually asymptomatic and secrete serous, mucoid, and rarely serosanguinous exudate. Polyps may be accompanied by potentially serious internal omphalomesenteric remnant such as Meckel's diverticulum attached to the umbilicus by obstructing fibrous bands.
Treatment
The condition is treated by surgical excision. However, underlying intestinal and urinary tract abnormalities are to be ruled out.
Umbilical Granuloma (Fig. 18)
It is basically a granuloma pyogenicum of the umbilicus. It presents as a red raw granulomatous growth over the umbilicus with purulent discharge.
Treatment
Treatment is cauterization with 75% silver nitrate solution, 20% potassium hydroxide or phenol.
“Knowledge has a beginning but no end.” –Geeta Iyengar
Umbilical Sepsis (Fig. 19A)
Umbilical sepsis, also known as omphalitis, is the inflammation of the umbilical stump of newborns. Most commonly it is attributed to bacterial infection. Normally the stump separates from the skin within 5–40 days after birth. A small amount of pus-like material is commonly seen at the base of the stump and can be controlled by keeping the stump open to air to dry. Certain bacteria can grow and infect the stump during this process and as a result significant redness and swelling may develop, and in some cases the infection can then spread through the umbilical vessels to the surrounding skin. Clinically, neonates with umbilical sepsis present with visible pus 12or grossly inflamed umbilical stump with history of high grade fever, vomiting, and poor feeding. It is an emergency and needs to be managed with promptness with oral/parenteral antibiotics and topical antibiotic cream/ointment. Cleaning of umbilical stump with antiseptics is also important.
Figs. 16A to E: (A) Neonatal varicella; (B) Neonatal varicella, note polymorphous lesions; (C) Neonatal varicella, note subsiding lesions; (D) Neonatal varicella, note subsiding lesions; (E) Shiny exudative lesion of umbilical polyp.
“Thinking is difficult, that's why most people judge.” –Carl Jung’
Congenital Syphilis [Figs. 19B(i) to B(vi)]
In congenital syphilis, the fetus becomes infected with the spirochete Treponema pallidum by way of placenta usually after 16th week of pregnancy. The clinical manifestations of early congenital syphilis (those occurring before 2 years 13of age) are anemia, fever, wasting, hepatosplenomegaly, lymphadenopathy, rhinitis, mucocutaneous eruptions, edema, desquamation, and pseudoparalysis. Of these, rhinitis or snuffles is the first sign to appear. It manifests between the 2nd and 6th week of life and is the result of an ulcerative lesion of the nasal mucosa. Deeper involvement of nasal cartilage can result in “saddle-nose deformity”. The cutaneous lesions are seen in one-third to one-half of infants affected by this disorder and appear as maculopapular or papulosquamous lesions most prominent on the face, trunk and extremities, diaper area, and palms and soles. The eruption generally develops slowly, as bright pink or red macules and papules and gradually fades to copper brown. It disappears spontaneously over 1–3 months leaving hypo- or hyperpigmented areas. The rare vesiculobullous, hemorrhagic lesions, when seen on the palms and soles, are highly diagnostic of this disorder. The palms and soles may be fissured, erythematous, and indurated. Desquamation of the skin in large dry flakes may occur over the entire body. Mucous membrane lesions, seen in one-third of the infants, are seen as weeping lesions or fissures at mucocutaneous junctions extending out from the lips in a radiating fashion over the skin. When deep, they leave residual scars (rhagades) in the adjacent circumoral region. Raised, flat, moist, wart-like lesions of condylomata lata are seen commonly over the anogenital region, around the nares, and at the angles of the mouth. About 90% of the infants show radiological evidence of osteochondritis and periostitis after the first month of life, most frequently affecting the long bones.
In late congenital syphilis, the disease persists beyond 2 years of age. It includes various signs and stigmata of congenital syphilis in infants in whom the diagnosis was overlooked or those who were inadequately treated early in the course of the disease. These include frontal bossing, saddle nose, short maxillae, and dental changes manifested in the form of Hutchinson's teeth (peg-shaped upper incisors), Moon's mulberry molars, gummas affecting the bones in the skull or tibia, syphilitic arthritis, paroxysmal cold hemoglobinuria and ocular changes such as retinitis and optic atrophy. Diagnosis is confirmed by serological testing and darkfield examination from the skin and mucous membranes.
“Happiness can be found, even in the darkest time, if one remembers to turn on the light”. –Albus Dumbledore
Treatment
Penicillin is the treatment of choice for all forms of congenital as well as acquired syphilis. Treatment should be started immediately after the diagnosis with aqueous crystalline penicillin G in a dosage of 100,000–150,000 units/kg administered intravenously every 8–12 hours 14or procaine penicillin G in dosage of 50,000 units/kg administered IM once daily for 10–14 days. Patients with congenital syphilis should have repeat quantitative, nontreponemal tests at 3, 6, and 12 months after treatment.
Figs. 19B(i) to B(vi): B(i) Red shiny scaly lesions over palms of congenital syphilis; B(ii) Congenital syphilis, similar lesions over soles; B(iii) Congenital syphilis, trunk; B(iv) Congenital syphilis, note the scaly papules; B(v) Congenital syphilis, close-up view; B(vi) Congenital syphilis.
“All our dreams can come true, if we have the courage to pursue them.” –Walt Disney
Subcutaneous Fat Necrosis (Figs. 20A to E)
It is a benign self-limiting dermatosis which affects apparently healthy full-term newborns and young infants. It presents as single to multiple sharply circumscribed areas of indurated and nodular plaques of skin over 15back, buttocks, thighs and shoulders, arms, and trunk. Its exact etiology is not known but proposed hypotheses are pressure on bony prominences during delivery, pre-eclampsia, obstetric trauma, fetal asphyxia or hypothermia, and neonatal infections. It is also seen in infants delivered by cesarean section and infants born to diabetic mothers. The onset of fat necrosis usually occurs in the first few days to weeks of life. The lesions at times may get calcified. However, most of the lesions undergo spontaneous resolution within few weeks to months. Complications include hypercalcemia, hypoglycemia, anemia, and thrombocytopenia.
Figs. 20A to E: (A) Close-up view of subcutaneous fat necrosis; (B) Erythematous indurated plaques of subcutaneous fat necrosis in a newborn; (C) Close-up view of the lesions; (D) Subcutaneous fat necrosis in a newborn with birth asphyxia; (E) Same child, another view.
“A ship is always safe at shore but that is not what it's built for.” –Albert Einstein
The condition needs to be differentiated from sclerema neonatorum which primarily occurs in newborns with sepsis, severe diarrhea, respiratory failure or dehydration and is a fatal condition with high mortality rate. There is woody induration of the skin and subcutaneous skin sparing palms, soles, and genitalia.
Management
Subcutaneous fat necrosis in most newborns resolves spontaneously and requires no specific therapy. Fluctuant lesions in cases of calcification should be aspirated to prevent their spontaneous rupture and secondary infection. In rare cases of hypercalcemia, restriction of calcium and vitamin D intake, systemic corticosteroids may be required. Use of furosemide diuretic, calcitonin and biphosphonates may be required in severe cases.
It is a diffuse, progressive, wax-like hardening of the skin and subcutaneous tissue that occurs in premature or debilitated infants during the first few weeks of life. In around 25% of cases, the mothers are ill at the time of delivery. Exposure to cold, peripheral chilling with vascular collapse, hypothermia, and an increase in the ratio of saturated to unsaturated fatty acids in the triglyceride fraction of the subcutaneous tissue have been hypothesized, but lack confirmation as possible causes for this disorder. This disorder is associated with underlying conditions such as sepsis, respiratory distress, dehydration or diarrhea, congenital heart disease and it is characterized by a diffuse nonpitting woody induration of the involved tissues. This disease usually starts on buttocks and legs and most of the time it progresses to involve all areas except palms, soles, and genitalia. When the disease spreads, the skin becomes mottled, cadaver-like, cold, stony hard, and yellowish white. At that time the face acquires a fixed mask-like expression and the limbs become immobile. The infants feed poorly, show clinical signs of shock, sluggish movements and in majority of cases the baby often dies.
Prognosis
Mortality occurs in 50–75% of affected children. Complications include sepsis, dehydration, electrolyte imbalance, respiratory distress, and gastrointestinal disease. The infants, who survive this disease, resolve without residual sequelae.
Management
There is no specific therapy for treatment of sclerema neonatorum. Supportive care with rewarming and administration of oxygen, control of infection, treatment of the underlying disorder, and intravenous therapy for correction of fluid and electrolyte imbalance are essential. Although indications for their use are not clear and controlled studies fail to confirm their efficacy, systemic corticosteroids in addition to antimicrobial agents have been advocated for infants with this disorder. Exchange transfusion has shown decreased mortality.
Hairy Pinna (Fig. 23)
It is a Y-chromosome mediated trait. It is considered as a pathognomonic sign in infants born to diabetic mother.
“Seek the wisdom of the ages, but look at the world through the eyes of a child.” –Ron Wild
17Polythelia (Supernumerary Nipples) (Fig. 24)
Supernumerary nipples are the remnants of the embryological milk line which runs from the anterior axillary fold to the inner thigh. Most often they are distributed over the anterior chest wall and upper abdomen as pink, umbilicated or elevated papules surrounded by a pigmented areola. A nipple may also be seen without areola and vice-versa. Usually there is only a single lesion, but occasionally multiple or bilateral nipples are present. It has been shown to be more common in male gender and on the left side. Accessory nipples have been reported to be associated with malformations of the urinary tract, in particular the kidneys. The association has been questioned recently.
Treatment
The accessory breast tissue may underlay the nipple and enlarge at puberty or in pregnancy. In such cases, complete excision is recommended because of the risk of malignant change.
Physiological Exfoliation of Newborn (Figs. 25A to C)
Neonatal desquamation is seen in 72–83% of newborns. This is mostly seen in postmature neonates. The exact pathophysiology of it is not known. In full-term infants, there is fine desquamation at birth within 24–48 hours and is localized to hands, ankles, and feet. There is widespread desquamation and cracking in postmaturity. Neonatal desquamation should be differentiated from collodion baby. However, there is no ectropion, eclabium or gloved appearance. The scaling gradually subsides over a period of 4–6 weeks.
Treatment
Application of oil and emollient (white soft paraffin) helps to reduce scaling and moisturize the skin of the babies.
Cutis Marmorata (Figs. 26A to C)
Cutis marmorata presents as reticulated bluish mottling of the skin seen on the trunk and extremities of newborn and infants. This phenomenon is a physiologic response to chilling with resultant dilatation of capillaries and small venules. It usually disappears as the infant is rewarmed. Although a tendency to cutis marmorata may persist for several weeks or months, this disorder bears no medical significance and no treatment is usually required. In some children, cutis marmorata may tend to recur until early childhood. In patients with Down syndrome, trisomy 18, and Cornelia de Lange syndrome, this reticulated marbling pattern may be persistent. In some infants, a white negative pattern of this phenomenon (cutis marmorata alba) may be created by a transient hypertonia of the deep vasculature. It is known as cutis marmorata alba. It is a transitory disorder and appears to have no clinical significance.
Treatment
No treatment is required for this physiological condition in newborns and infants. Reassurance and counseling of the parents is important.
“Many of life's failures are people, who did not realize how close they were to success when they gave up.” –Thomas Edison
Cutis Marmorata Telangiectatica Congenita (Fig. 26D)
It is a condition which closely simulates cutis marmorata but does not disappear on rewarming. The condition presents at or shortly after birth. The pigmentation 18presents as red marbled mottled patches. The changes may be limited to a localized part of the body. The skin changes get accentuated by decrease in ambient temperature. Contrary to cutis marmorata or livedo reticularis, there may be atrophy or ulceration of the skin. There may be associated limb hypoplasia, hyperplasia or vascular abnormalities, e.g., port-wine stain, Sturge-Weber syndrome, etc. Various associated neurological disorders include seizures, hydrocephalus, and ocular abnormalities such as glaucoma, retinal pigmentation, and retinal detachment. A condition named Adams-Oliver syndrome is characterized by cutis marmorata telangiectatica congenita (CMTC) with aplasia cutis congenita and distal transverse limb defects.
Figs. 25A to C: (A) Physiological exfoliation of newborn; (B) Close-up of neonatal exfoliation; (C) Note exfoliation over thumb and fingertips.
Within first 2–3 years of life, CMTC gradually improves on its own. When the lesions are present over face, ophthalmological evaluation is necessary. When there are neurological symptoms, referral to a neurologist is necessary.
Neonatal Irritant Dermatitis (Figs. 27A and B)
Irritant dermatitis can develop within 24–48 hours of exposure to irritant chemicals. In newborn babies often various antiseptic cleansers and lotions cause dermatitis of the soft and sensitive skin. A severe form of irritant dermatitis can result in burn in neonatal intensive care unit (NICU) setup either from phototherapy, heater, or undiluted antiseptics.
Diagnosis
Intense erythema with formation of papules and vesicles over the areas of contact with the offending agents is seen. The newborn baby becomes restless and irritable.
“Strive not to be having success, but rather to be of value.” –Albert Einstein
Treatment
Avoidance of offending agent should be prompt. Cleansing of skin with distilled water or normal saline is to be done.19
Figs. 26A to D: (A) Cutis marmorata telangiectasia in a newborn baby; (B) Same baby, note net-like telangiectasia; (C) Cutis marmorata, close-up view; (D) Cutis marmorata telangiectatica congenita.
Figs. 27A and B: (A) Acute irritant dermatitis, note glazed erythema; (B) Irritant dermatitis over back.
“Don't count the days, make the days count.” –Muhammad Ali
Application of emollients such as white soft paraffin 2–3 20times a day for 7–10 days clears the dermatitis in most of the newborn babies. Occasionally mild topical steroids, e.g., 1% hydrocortisone or clobetasone butyrate may be required.
Epstein's Pearls and Bohn's Nodules
Clinically these are counterpart of facial milia. These appear as multiple, discrete 2–3 mm pearly white or yellowish papules over midline of hard palate (Epstein's pearls) or gum margins (Bohn's nodules). They are seen in 64–94% of normal neonates, but less commonly affects the premature neonates.
Diagnosis
The characteristic appearance and location clinch the diagnosis.
Treatment
It is a physiological condition in newborns and usually disappears in 4–6 weeks time is to be explained to the parents.
Sebaceous Gland Hyperplasia (Fig. 28)
It manifests as multiple, tiny, pin-point yellowish papules distributed on the nose, upper lips, malar region, and chin. It is due to influence of maternal androgens. They are rarely seen in premature infants.
Diagnosis
Diagnosis is based on its characteristic appearance and site.
Treatment
Reassurance of anxious mother.
Transient Neonatal Pustular Melanosis (Figs. 29A to C)
It is a benign self-limiting disease of unknown etiology. This rare condition is mostly seen in darker skin. It is seen in less than 1% infants.
Diagnosis
Small sterile superficial vesiculopustules develop which rupture easily within 24–48 hours and evolve in to hyperpigmentation. The commonly affected areas are forehead, area below chin, neck, lower back and shins, but can become generalized. There is no systemic involvement. Though the lesions usually disappear within 2–3 days, hyperpigmentation lasts for 3 months.
Figs. 29A to C: (A) Transient neonatal pustular melanosis; (B) Transient neonatal pustular melanosis, note hypopigmented macules suggesting healed pustules; (C) Close-up view of transient neonatal pustular melanosis.
“If you cannot fly, then run; if you cannot run, then walk; if you cannot walk, then crawl but whatever you do, you have to keep moving forward.” –Martin Luther King JR
No treatment is required as it is a self-limiting condition.
Acropustulosis of Infancy
Acropustulosis of infancy is an idiopathic noninfective pustulosis affecting infants and small babies. Usually, it occurs between 2 months and 3 years of age. The lesions appear as recurrent crops for few weeks to months and subside by 3–4 years of age. Though the exact etiology is not known, a possible association with preceding scabies has been implicated.
Diagnosis
The lesions typically appear as pinpoint erythematous papules which transform into pustules within 24–48 hours. Palms, soles, dorsa of hands, and feet are the common sites affected. Usually it is associated with moderate-to-severe pruritus.
Differential Diagnosis
Various closely simulating conditions are scabies with secondary infection, impetigo, dyshidrotic eczema, erythema toxicum neonatorum, neonatal pustular melanosis, etc.
Treatment
Moderately potent topical corticosteroids such as mometasone or fluticasone may be used for 2–3 weeks. In severe cases, dapsone (2 mg/kg body weight in two divided doses) has also been used. Systemic antihistamines can be given for pruritus. Recently topical maxacalcitol has shown to improve the symptoms.
Infantile Gluteal Granuloma (Figs. 29D and E)
Infantile gluteal granuloma, also known as granuloma gluteale infantum, is a rare, benign nodular complication of irritant diaper dermatitis in infancy due to use of potent topical corticosteroid, prolonged occlusion from diapers resulting in increased exposure to feces and urine and secondary infection with candidiasis. It is seen more commonly in the age group of 4–9 months.
Diagnosis
The disease characteristically presents as multiple erythematous to violaceous papulonodules over the gluteal region, external genitalia, inner thighs, and lower abdomen in infants.
Treatment
Stoppage of topical steroid application is the first thing to be done. If there is any evidence of candidal infection, topical ketoconazole or clotrimazole should be prescribed. Otherwise, the lesions subside very slowly without any treatment over a period of 3–13 months. Reassurance of parents and their counseling is of paramount importance.
“Opportunity is missed by most people because it is dressed in overalls and looks like work.” –Thomas Alva Edison
Trichostasis Spinulosa (Fig. 29F)
It is a common disorder characterized by comedone-like papules which represent horny plug with 25–50 telogen vellus hair embedded within it. The basic defect is infundibular hyperkeratosis of hair follicle which prevents shedding hence retention of telogen vellus hair originating from a single hair matrix. This entity can be classified into two variants. Classically the disease presents as comedone-like papules resembling black head distributed 22mainly over nose and forehead in elderly individuals. Other variant characterized by pruritic follicular papule resembling keratosis pilaris is distributed over the trunk and extremities of young individuals or newborns. Pathological condition commonly associated with this entity is renal failure. Diagnosis can be established with the use of dermoscope and microscope. Various treatment modalities are depilation, keratolytic, topical and systemic retinoids, and hydroactive adhesive pads. Pulsed diode laser shows encouraging result.
Aplasia cutis congenita (ACC) is a congenital defect of unknown etiology characterized by congenital absence of skin in newborn babies. Histologically, there may be absence of either epidermis, dermis or subcutaneous tissue. It usually affects the scalp but face, trunk or extremities can also be involved. Most of the cases of ACC are sporadic. Familial cases have been reported. Antithyroid drug methimazole given to a pregnant mother is likely to produce ACC in newborn babies. However, the issue is far from being conclusive.
Diagnosis
Aplasia cutis congenita classically presents as single or multiple, oval to circular sharply demarcated granulating ulcerations over scalp. The size of the lesions usually varies from 1 to 3 cm. Occasionally, lesions may look like either a membrane or keloid.
Associations
Although most infants with ACC are otherwise well, various associated anomalies reported are cleft lip, cleft palate, hemiatrophy of limbs, ocular abnormalities, gastrointestinal malformations, spinal dysraphism, hydrocephalus, seizures, mental retardation, trisomy 13, vascular anomalies, etc. ACC is associated with fetus papyraceus (vanishing twin syndrome).
Figs. 30A to D: (A) Aplasia cutis congenita (ACC) at birth; (B) ACC in a newborn; (C) Close-up view of ACC, note prominent visible blood vessels; (D) Hairless patch of ACC over scalp.
“And in the end, it's not the years in your life that count; it's the life in your years.” –Abraham Lincoln
Forcep injury and other types of birth injury need to be differentiated.
Figs. 31A(i) and (ii): A(i) Aplasia cutis congenita in a newborn; A(ii) Aplasia cutis congenita at birth in a mother on methimazole during pregnancy.
Treatment
By and large ACC with small defects (up to 3 cm) do not require any treatment. Proper cleansing of the area daily with betadine is required in cases of granulating lesions. Prevention of secondary infection is very important. Most of the lesions heal with scarring by 6 weeks to 6 months and occasionally by 12 months. The scars gradually become less conspicuous over next 4–5 years. However, for bigger defects (>3 cm), plastic surgery is advisable.
Venous Prominence Over Bridge of the Nose
This physiological condition is seen in 1 in 10,000 live births. A transverse superficial vein remains prominent over the bridge of the nose. This condition is usually seen in 3–9 months old babies. Subsequently it disappears. Similar prominent veins are seen over the chest of babies admitted in NICU with respiratory distress.
Treatment
Explaining the condition and its benign self-limiting nature to the anxious mother is all that is necessary.
Neonatal Erythroderma (Figs. 31B to F and 32)
Erythroderma is defined as any inflammatory condition involving more than 90% of body surface area. Neonatal erythroderma, also called as “red scaly baby,” is a life-threatening entity seen at birth or during the first 1 month of life. Neonatal erythroderma can be part of genodermatosis, immune deficiency, inflammatory disorder, metabolic diseases, and infections. Atopic dermatitis presenting as erythroderma is usually observed later and hence not a common differential for neonatal exfoliative dermatitis. Various causes of neonatal erythroderma are:
Cutaneous disorders:
- Infantile seborrheic dermatitis
- Atopic dermatitis
- Psoriasis
- Pityriasis rubra pilaris
- Diffuse cutaneous mastocytosis
- Ectodermal dysplasia (hypohidrotic/anhidrotic)
Ichthyosis:
- Lamellar ichthyosis
- Nonbullous ichthyosiform erythroderma
- Conradi–Hünermann syndrome
- Bullous ichthyosiform erythroderma
- Netherton syndrome
Infections:
- Staphylococcal scalded skin syndrome
- Toxic shock syndrome
- Congenital cutaneous candidiasis
Immunodeficiency:
- Omenn syndrome
- Graft-versus-host reaction
Metabolic disorders:
- Disorders of biotin metabolism
- Essential fatty acid deficiency
- Multiple carboxylase deficiency
- Acrodermatitis enteropathica
- Leiner's disease
Drugs:
- Ceftriaxone
- Vancomycin
“The good life is one inspired by love and guided by knowledge.” –Bertrand Russell
Infantile seborrheic dermatitis (ISD) which manifests in the neonatal period, usually presents with greasy scales on the scalp (cradle cap), skin folds such as the axilla, neck, retroauricular, and diaper areas. Atopic dermatitis may have its onset in the first month; however, it is rarely erythrodermic in neonates. The lesions usually are vesicular and exudative in nature. Sometimes there is a significant overlap between infantile seborrheic dermatitis and atopic dermatitis.
Figs. 31B to F: (B) Neonatal erythroderma; (C) Note scaling and glazed erythema all over body; (D) Appearance justifies nomenclature of neonatal erythroderma as “red baby”; (E) Close-up view of “red baby”; (F) Another view of “red baby”, close-up.
“Failure is simply the opportunity to begin again, this time more intelligently.” –Henry Ford
Neonatal psoriatic erythroderma is a rare entity and it commonly presents as recalcitrant diaper dermatitis which may become generalized with a widespread pustular form 25of the disease. This is accompanied by periodic high fever, and the child becomes very toxic with recurrent crops of superficial pustules appearing on erythematous plaques. Psoriasis and pityriasis rubra pilaris may look similar with erythematous scaly plaques which may enlarge and become generalized to produce erythroderma. Heavy infiltration of the entire skin with mast cells results in diffuse cutaneous mastocytosis. The skin undergoes lichenification and is erythematous, producing urtication and hemorrhagic bullae formation on mild trauma. Diarrhea, flushing, and respiratory symptoms are the common accompaniments. Darier's sign, a wheal which flares on stroking the skin, is often positive. Although a life-threatening condition, it improves with age.
Several varieties of ichthyoses can manifest as erythroderma. Patients of nonbullous congenital ichthyosiform erythroderma have finer scales and are more inclined to develop erythroderma. The newborns have a collodion membrane which desquamates, revealing the erythroderma. It fades in mild disease but in the severe classic form, large plate-like scales with erythema persists. Harlequin ichthyosis infants with hyperkeratotic fissured plates often die due to respiratory problems; if they survive with treatment, they manifest generalized erythroderma. Epidermolytic hyperkeratosis or bullous ichthyosis has widespread denuded areas which resolve slowly, manifesting underlying erythroderma. Netherton syndrome presents with features of generalized erythroderma, fragile hair with trichorrhexis invaginata (“bamboo hair”), and atopic diathesis such as severe rhinorrhea, asthma, anaphylaxis due to food, and increased serum IgE levels. Erythroderma at birth is often the onset and it is a diagnostic dilemma where sparse hair with shaft defects may take several microscopic examinations, especially of eyebrows and lashes, before clinching the diagnosis. These patients are atopic and often have intercurrent infective episodes with high rates of mortality. The serum IgE levels are markedly raised and are even more than seen normally in atopic subjects.
“Everything that we ask about others can lead us to an understanding of ourselves.” –Carl Jung
Because of the protective effect of maternal immunity, congenital immunodeficiency syndromes are rarely symptomatic at birth. Graft-versus-host reaction from 26maternal engraftment can, however, occur even during intrauterine development. Omenn syndrome is a familial reticuloendotheliosis with eosinophilia having erythroderma, failure to thrive, lymphadenopathy, and recurrent infections. Marked leukocytosis, eosinophilia, anemia, and hypogammaglobulinemia are some of the findings in this histiocytic disorder. Hypogammaglobulinemia can start with diarrhea and periodic fever together with erythroderma. Dermatitis begins by 4 weeks of age and rapidly generalizes. DiGeorge syndrome and severe combined immunodeficiency may also present with eczematous dermatitis leading to erythroderma.
Graft-versus-host reaction may occur in T-cell immunodeficiency or as a result of transplacental transfer of maternal lymphocytes as a sequela of exchange transfusion. An erythematous nonspecific morbilliform rash may lead to erythroderma and epidermal sloughing. Neonatal cutaneous T-cell lymphoma can present with congenital ichthyosis with atypical Sezary-like lymphoid cells in skin and lymph nodes and other immunological abnormalities.
Metabolic and nutritional disorders are suspected when the infant has failure to thrive and the dermatitis manifests periorificially at the onset before it generalizes. Severe protein malnutrition during infancy can present with widespread erythema, edema, erosion, and desquamation of the skin. Deficiency of zinc due to malabsorption as in acrodermatitis enteropathica or low concentration of zinc in breast milk can begin with psoriasiform dermatitis in circumoral or periorificial areas which may crust and spread to involve other areas; this has also been reported in children with acquired immunodeficiency syndrome. Diarrhea, failure to thrive, irritability, and photophobia can accompany such dermatitis. Essential fatty acids, mostly found in dairy products and vegetable oils, are supplemented in the diet. Diffuse desquamation, lichenification, and intertriginous dermatitis can develop in such situations. Cystic fibrosis dermatitis presents with psoriasiform diaper rash not responsive to topical steroids or antifungals. This dermatitis may spread and is associated with growth failure and irritability. Deficiency of holocarboxylase synthetase manifests with neonatal erythroderma. The children have alopecia, secondary cutaneous candidiasis, dehydration, and ketoacidosis which can lead to a fatal outcome. Deficiency of holocarboxylase synthetase in skin fibroblasts clinches the diagnosis. Deficiency of biotinidase presents with patchy alopecia and acrodermatitis enteropathica-like skin lesions. These conditions encompass the group of “multiple carboxylase deficiency” disorders. Penicillins, aminoglycosides, and cephalosporins often produce erythematous maculopapular skin lesions, but rarely erythroderma. Ceftriaxone and vancomycin in neonates may produce erythroderma, and vancomycin may also induce hypotension on account of histamine release.
Leiner's disease is literally a clinical phenotype of acquired erythroderma, diarrhea, and failure to thrive. Desquamative generalized erythema and dermatitis with weight loss was thought to be common in breast-fed infants due to the deficiency of biotin. Cases of generalized dermatitis in seborrheic pattern due to immunodeficiencies were also included in this category.
Laboratory investigations are supportive to clinical diagnosis in neonatal erythroderma. Histopathology is not diagnostic in all conditions.
Treatment
Managing neonatal erythroderma is a therapeutic challenge as it is very difficult to treat this potentially life-threatening situation. Careful monitoring of the vital signs, maintenance of fluid and electrolyte balance, and prevention of hyperpyrexia are mandatory in the management. Application of emollients, wet dressings, topical steroids, and systemic antibiotics are the other modalities. Maintaining the skin barrier and proper hydration is the key to managing most of the conditions. Oral retinoids (acitretin) in dose of 0.5–1 mg/kg is indicated in congenital ichthyosis. Regular follow-up and prevention of complications reduce mortality in neonatal erythroderma.
Amniotic Band Syndrome (Figs. 33A and B)
Amniotic constriction bands are congenital anomalies characterized by constriction rings and digital amputation of one or more digits or limbs. The amniotic bands are confined to the skin, soft tissue and may extend deep into the tissue. It results from intrauterine rupture of amnion with formation of fibrous bands encircling the fetal parts resulting in constriction of the underlying tissue. The inheritance is sporadic. Constrictions can occur on limbs giving rise to swelling on the distal part, loss of distal part of one or more fingers, toes, contractures, and fracture. They can be associated with craniofacial and spinal defects. Rarely deformation of internal organs can occur.
“It is the province of knowledge to speak and it is the privilege of wisdom to listen.” –Oliver Wendall Holmes
Congenital Erosive and Vesicular Dermatosis (Figs. 34A to D)
The condition presents as vesicles and ulceration in newborns, almost exclusively in premature babies. 27The exact cause is not known but intrauterine infection and amniotic membrane adhesion are proposed hypothesis. Essentially it is a nonhereditary condition.
Figs. 34A to D: (A) Congenital erosive and vesicular dermatitis; (B) Congenital erosive and vesicular rash on healing; (C) Congenital erosive and vesicular dermatitis; (D) Healed pigmentary changes in congenital erosive and vesicular dermatitis, note the soft supple and reticulate scarring.
“Knowledge will give you power, but character respect.” –Bruce Lee
Diagnosis
Extensive vesicles and erosions in a reticulate pattern over extremities, trunk involving up to 75% of body 28surface during first month of life in premature babies is the usual presentation. The lesions soon get crusted and subsequently heal with distinctive supple and reticulate scarring. The scars over the trunk have a cobblestone-like appearance. There may be associated scarring alopecia and ulceration over the tongue. Nails may be either absent or hypoplastic. Sweating is absent over the scarred areas and the baby may present with hyperthermia. Rarely neurological and ocular abnormalities may be seen.
Differential Diagnosis
The condition needs to be differentiated from epidermolysis bullosa. Lack of involvement of face, hands and feet, characteristic rippled scar on healing and progress of lesions characterize the condition and differentiates it from epidermolysis bullosa.
Treatment
It is symptomatic and essentially care of the wound. Reassurance of parents is extremely important.
Harlequin Color Changes (Fig. 35)
Harlequin color change is a rare physiological phenomenon seen more commonly in preterm, low birth weight, sick infants, and with severe intracranial injury. It can affect 10% full-term infants. It is probably related to immaturity of the hypothalamic centers and autonomic control of vascular tone leading to capillary bed dysregulation. The color change occurs when the child is lying on one side; the dependent side becomes strikingly red with simultaneously blanching on the upper half of the body with a sharp line of demarcation. The mucosa and genitalia are spared. These episodes may persist for few seconds to minutes (30 sec to 20 min). The color change is rapidly reversible with a change in position, crying, and increased muscular activity. The onset of this phenomenon is seen at 2–5 days of age and may last up to 3 weeks, but can present within first few hours of birth. Repeated episodes can be present in 50% of the affected neonates. Other associated conditions that can trigger this phenomenon are meningitis, seizures, and tricuspid atresia.
Treatment
The condition needs to be explained to the parents and reassured.
Figs. 36A to C: (A) Median raphe cyst; (B) Median raphe cyst, note beaded whitish papules; (C) Median raphe cyst, note beaded whitish papules along with ulcerated papule.
“Success is a lousy teacher. It seduces smart people into thinking they can't lose.” –Bill Gates
Median Raphe Cyst (Figs. 36A to C)
Median raphe cyst (MRC) is a rare, congenital, benign lesion that can occur anywhere along the ventral side of median raphe of male external genitalia from anus to urethral meatus. MRCs are usually asymptomatic during childhood. They become symptomatic during adolescence or adulthood following vigorous sexual intercourse. Proximal location and small size, <2 cm are 29also contributory to their asymptomatic nature. Distal location and bigger size lesions are usually symptomatic. Patients may present with pain, difficulty in voiding, difficulty in intercourse, hematuria or hematospermia. Location of MRC varies widely along the ventral midline of the perineum from anus to urethra. They do not connect with urethra. The most common location is distal penile shaft and parameatal position. Rarely do they present at scrotum, glans penis, and prepuce. Sometimes they may present as cord or series of beads (canal-like). Clinical differentials include epidermoid inclusion cyst, dermoid cyst, steatocystoma, glomus tumor, and apocrine cystadenoma. Pathogenesis is not fully elucidated. They may arise from defective fusion of urethral fold leading to tissue trapping during development of urethral fold. Developmental anomaly of periurethral gland of Littre or blockage of paraurethral ducts could be other possibilities. Lining of the cyst may vary depending upon tissue trapping. It could be pseudo stratified columnar epithelium (if proximal urethral cells get trapped), squamous epithelium (distal urethral cell), and glandular epithelium (periurethral gland get trapped). Ciliated cells are rarely present. Metaplastic change due to local irritation is the most probable explanation. Treatment is surgical excision for cosmetic reason or for symptomatic lesion. Simple aspiration may lead to recurrence therefore wide local excision and aspiration is probably the better way to approach.
Figs. 37A to C: (A) Sternal cleft with umbilical raphe in a newborn; (B) Sternal cleft with umbilical raphe in a newborn, close-up view; (C) Milder form of the defect.
“The whole problem with the world is that fools and fanatics are always so certain of themselves, and wiser people so full of doubts.” –Bertrand Russell
Sternal Cleft with Umbilical Raphe (Figs. 37A to C)
Sternal cleft is a rare congenital malformation that can be isolated or combined with other congenital defects. It is presumed to be caused by failure of fusion of sternal bands during the sixth to ninth weeks of gestation. Studies in mice lead to the identification of impairment in the expression of Hoxb genes as a possible cause of sternal defects. The malformation is usually sporadic, but there are reports of familial inheritance (autosomal dominant). 30They can be classified as superior, subtotal, total, and inferior. Superior sternal clefts are usually isolated but may be associated with vascular abnormalities such as craniofacial hemangioma (seen almost exclusively in females) and supraumbilical midline raphe extending from the umbilicus to the lower part of the sternal defect, first described by Leiber. Coarctation of the aorta with a right aortic arch and congenital aortic aneurysm has also been described in association with cleft sternum and supraumbilical raphe. PHACE syndrome (posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies, and eye anomalies) is sometimes associated with ventral developmental defects (sternal cleft, supraumbilical raphe, or both) and is then called as PHACE(S) syndrome.
It may be an abortive form of Pentalogy of Cantrell. Pentalogy of Cantrell is a condition characterized by a combination of midline birth defects that can potentially involve the breastbone (sternum); the muscle that separates the chest cavity from the abdomen and aids in breathing (diaphragm); the thin membrane that lines the heart (pericardium); the abdominal wall; and other vital organs.
Prenatal high-resolution ultrasonography may diagnose the condition and helps to accurately evaluate cartilaginous parts of the sternum and possible cardiovascular malformations postnatally. Surgical correction during the neonatal period is recommended as the compliant thorax allows direct approximation of the sternal halves.
CARE OF THE NEWBORN SKIN
The skin is the first barrier of the newborns to counter various noxious factors/agents of the environment once the baby comes out of the safe and secured intrauterine life to the external world. Various aspects of newborn skin care will be discussed under different headings.
Need for Special Skin Care for Babies
Baby's protective but delicate cover needs to be kept in a healthy condition and it should be disturbed as little as possible. Topical agents are more rapidly absorbed into infant skin due to deficient intercellular bridges. Besides, greater body surface area to weight ratio than adults also facilitates easy absorption and toxicity of topically applied substances. Infant skin cannot withstand the toxicity of most substances as they make this delicate skin more susceptible to electrolyte imbalance, fluid imbalance, and thermal instability. Infant skin is particularly very sensitive to cleansing agents as they contain stronger chemicals and may be drying. Hence, the product should be guaranteed of safety for use on babies.
Skin problems pertaining to dryness are common in babies due to inadequacies in the epidermal barrier. The skin irritation potential toward topical applicants is also more. Maintaining skin integrity and preventing exposure to toxic substances in childhood assures healthy skin for several years ahead.
Skin of the newborns performs the most challenging task as it is the outermost cover of the body. Moreover, it is confronted with various factors, namely, temperature changes, friction, microbes, etc., of the external world once the baby is born. As the structure and functions of the skin depend on whether a child is born at term or prematurely, skin care is related to gestational age.
At birth, microbial colonization of newborn skin is almost nil. But over a few hours, aerobic flora occupies skin at different concentrations at different sites, e.g., mostly over groins, axillae, and scalp. Coagulase negative staphylococci (Staphylococcus epidermidis) are the most commonly found microorganisms. Staphylococcus aureus appears only as contamination, usually from mother or nursing staff.
Skin Care at Birth
Removal of Vernix Caseosa
At birth the baby's skin is coated with vernix caseosa, blood, meconium, and cellular debris. Vernix caseosa has a protective function, contains both epidermal (triglycerides and cholesterol) and sebaceous (squalene and waxes) fat. Premature infants tend to have less of vernix than term babies and postmature babies have little vernix. There is considerable interindividual variation in the quality of vernix caseosa. Vernix caseosa should not be rubbed or removed as it dries and falls itself by 5–7 days.
Washing and Bathing
A bath is an ideal means of cleaning an infant completely. A bath in infant should not last for more than 5 minutes. The bath of approximately 5 minutes increases the hydration of skin and, thereby, reduces the threshold for friction. Newborn temperature should be stabilized before the first bath is given. Bathing should be deferred till fifth or seventh day. Sponge bathing can be given till the cord falls. Blood and meconium can be wiped with soft cotton dipped in warm water. Temperature of bath water should be equal to skin temperature (34–36°C).
“It's amazing what you can get, if you quietly, clearly and authoritatively demand it.” –Meryl Streep
31A solid or liquid cleanser or a syndet can be used to clean baby skin. After bath, infants must be dried thoroughly, particularly over skin creases, groins, and axillae.
Diaper Care
Napkin should be changed frequently every 2 hours, at least at each nursing and feeding time. Cloth diaper should be carefully washed in lukewarm water and then rinsed off and dried thoroughly.
The diaper area is specifically vulnerable because it is a closed environment suitable for microorganisms and with frequent wetting, it is more often moist; hence the skin becomes prone to maceration and increases its permeability to other irritants.
The skin here is constantly in contact with strong alkalinizing agents, e.g., urine and feces and the pH here is prone to high alkalinity that damages the skin integrity. It is, thus, very important to be well-aware of the need to change nappies and the range of products that are available to prevent any rash or irritation in the nappy area.
Nappy rash can be reasonably prevented by reducing moisture by the frequent changing of nappies. This would reduce contact between urine, feces, and the skin. However, this does not seem feasible at most instances. In such cases, using partially occlusive agent such as petrolatum or zinc-based products on the genital area can help to form a physiological barrier that minimizes this interface. As far as possible, air exposure should be increased by allowing the child to move around the house bare-bottomed. Plastic pants should be avoided as they reduce the air circulation to the skin. Warm water and soft cotton wool should be used to wipe the nappy area. Feces have a tendency to stick and scrubbing only worsens the status of the delicate skin. Here, the use of an emulsion such as baby lotion can ease the removal by reducing the surface tension and cleaning the debris. Skin should then be thoroughly dried each time the diaper is changed by exposing it for a few minutes. The bottom should be wiped from front to back to avoid fecal matter from reaching the genitals. Soaps should be mild and should be used very rarely if a rash has developed. Use of powders in diaper areas is not recommended.
In general, the nappies should be made of cotton cloth and should be home laundered with mild detergents. Recent advances in diaper technology have led to decrease in frequency and severity of diaper rash. Diapers with highly superabsorbent acrylate gel can be used.
Scalp
Regular cleansing of scalp is necessary. Shampoo helps to remove scales and crust from the scalp (cradle cap). Coconut oil or mineral oil application softens the scales.
Nails
Nail should be regularly trimmed and kept short and clean.
The Ears
Outer aspect of ear should be cleansed. Cotton swabs soaked in boiled water should be used to clean ears. Special care should be taken not to hurt auditory canals.
Umbilicus
After birth umbilical cord dries out and drops off within 5–10 days. No topical agents should be used on umbilical cord. Dry cord care is recommended. Prophylactic use of chlorhexidine reduces the colonization and prevents sepsis.
Skin Care in the Premature Infants
After birth, skin maturation proceeds rapidly in preterm infants. These infants are kept warm and nursed in closed incubators. Environmental conditions in these units are potentially harmful for infant skin, which is subject to scarring. Cosmetically or functionally, significant lesions may be caused by needle marks, central venous catheters, transcutaneous oxygen monitoring, chest drain insertion, extravasation of intravenous fluid (Fig. 38) or skin stripping due to adhesive tape. To reduce the frequency and severity of skin damage, neonatal staff need to know that many routine procedures can lead to long-term scarring and atrophy. Heaters should be kept at a safe distance in NICU as there is a risk of burn injury (Fig. 39).
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- Disinfection: The most common infective agents causing septicemia are coagulase-negative staphylococci in relation to catheter placement. For prevention, maintain hygiene by hand washing of the staff and parents. Cleaning with chlorhexidine-alcohol and povidone-iodine, two consecutive 10-second cleaning destroys more than a single 10-second wipe.
- The incubator: Frequent change of infants’ position in the incubator reduces the risk of skin erosion and impending bed sore. Fingers and toes must be kept visible. Catheters or needles should be secured with a transparent tape to allow easy detection of fluid extravasation. Scarring alopecia can develop following pressure ulcer. The occurrence of nonblanchable erythema and disruption of epidermis indicate impending ulcer. The occurrence of scarring alopecia has been reported in infants from pressure necrosis.
- Transcutaneous oxygen monitors: Transcutaneous oxygen monitors should not be left in place for more than 1 hour without surveillance. Nonblanchable erythema has been reported with keeping such electrodes for prolonged period. The use of karaya electrodes has been demonstrated to be effective in cardiorespiratory monitoring with decreased trauma to the neonatal skin. Placement of electrodes on the limbs, especially in very low birth weight infants, can eliminate the need to frequently remove these pads to facilitate auscultation or other assessment of the chest wall.
- Minimal use of tape and adhesive: The skin of the premature infants may be damaged by repeated attachment and removal of adhesive tapes to secure electrodes, IV cannulas, drains, etc. Adhesives should be used on small areas of skin and removed gently with warm water soaked gauze and diluted soap, but not alcohol, which may be irritant for baby's skin.
- Emollients: Application of emollient is a safe and effective way to decrease neonatal peeling and scaling dermatitis. Coconut oil or vegetable oil applied gently to the skin, reduce scaling and fissuring as well as increase skin hydration.
Skin Care of the Term Baby and Infant
The large number of infant skin-care products available over the counter is at times confusing for the average consumers. These have been the gifts of media and so-called health magazines for the lay people with all rosy advertisements. By and large the principle followed by a doctor should be to advice the parents to go for a product marketed by a multinational company or a company of good repute and stature or a product, which has been in the market for a considerable period of time and thus has stood “the test of time”. Several types of products are used, namely soaps, shampoos, antiseptics, moisturizers, etc.
Detergents
The term “detergent” designates a substance capable of cleaning the skin, i.e., of removing impurities (dust, grease organic secretions, microorganisms). Washing with water alone does not remove all the impurities on the skin surface. Some are only fat soluble, thus requiring the use of products capable of emulsifying the fatty substance into fine droplets, which can then, be carried away by rinsing. These products are known as surfactants, act by suppressing the surface tension, which allows fatty substances to remain on the skin surface. Detergents act by reducing the surface tension between water and air, creating a foaming effect not directly correlated with the cleaning properties of the product. As a rule, a higher foaming power increases the risk of damage to the skin. Detergents are classified as ionic or nonionic products. In infants detergents should be used cautiously, followed by a thorough rinsing. Too much removal of lipids from the stratum corneum would eliminate those essential to the surface ecosystem.
Soaps are the products of saponification, i.e., the action of alkali on a fatty substance. In hard water, soaps tend to precipitate.
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Syndets or synthetic detergents do not have the theoretical disadvantages of soap but are subject to rapid disintegration. They can produce excessive dryness of skin, if moisturizers are not added to it. Antiseptic soaps are useful in preparation of an operative field, but are 33unsuitable for daily use in infants as they can cause irritation to infants’ skin. Moreover, antiseptic soaps remove the commensal organisms from the skin surface, thereby, making the skin prone to attack by virulent pathogenic organisms from outside.
Bubble bath products attract the infants and their parents because of its colorings and perfumes and, thereby, mask the risk of prolonging a bath and irritating the genital mucosa.
Most baby shampoos in the market contain anionic surfactant which ensures adequate cleansing. The pH should be close to that of tears and, thereby, would not cause irritation to the eyes. Special ingredients, e.g., selenium sulfide, ketoconazole or zinc pyrithione may be added to the shampoos for seborrheic dermatitis or seborrhea scalp. The basic principles of use of various antiseptics and emollients in term babies and infants are essentially the same as in preterm babies.
Protective creams: These are basically prepared to reduce the risk of irritation, particularly napkin rash, by isolating skin from numerous irritants for baby's skin. The creams contain a fatty phase, an aqueous phase, a surfactant, additives (zinc oxide), scents, and preservatives. These creams can paradoxically cause increased occlusion and irritant dermatitis to its ingredients.
Powders: Application of powders over the skin folds is not recommended.
Role of Massage
The act of touch fulfils the basic need to feel safe, comfortable and loved. Touch is also an intrinsic factor in child development. Touch is proposed to play a role in growth, development, and overall well-being. Massage is one of the most beautiful and gentle methods of touch. It is practiced in most countries and has recently been researched extensively in western countries. Indian form of infant massage is appreciated all over the world. It has been seen that massage with oil is more beneficial as compared to massage without oil. It is important to note that the oil used in such a situation ought to be smooth, of optimum viscosity, and friction free or else it would lead to abrasions on the skin surface. The oil should be nonocclusive so that it does not block the skin pores and allows the skin to breathe. It ought to be safe and mild to suit the baby's delicate skin and the ingredients should be thoroughly tested for their potential to cause contact sensitivity. Mineral oil is one of the best-known moisturizing ingredients ever found. It spreads easily and has a long-lasting tactile effect, making it an extremely efficacious emollient. Omission of low molecular weight hydrocarbons alleviates risks of carcinogenicity while the large particle size renders it incapable of blocking pores making it noncomedogenic.
Massage should be started after 1 month and can easily continue till 10 years and over. Benefits of massage are numerous. Appropriate knowledge of correct massage techniques is important in order to attain maximum therapeutic benefits from it. Complete head-to-toe massage should be a daily routine. But massage should be gentle and judicious. However, massage should be withheld for a few days in case either the baby has got any contagious infection of skin or the person offering massage has any infection over hands. Massage given by the mother increases the bondage between the mother and her baby. It helps in the physiological and psychological development of the babies.
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SUGGESTED READING
- Bayliss SJ, Colven R. Disorders of subcutaneous tissue in newborn. In: Irvine AD, Hoeger PH, Yan AC (Eds). Harper's Textbook of Dermatology, 3rd edition. Wiley-Blackwell; 2011. pp. 7.1–7.5.
- Eichenfield LF, Frieden IJ, Esterly NB. Neonatal Dermatology, 3rd edition. Philadelphia PA: Saunders Elsevier; 2015.
- Gorur DK, Murthy SC, Tamraparni S. Early neonatal dermatoses: a study among 1260 babies delivered at a tertiary care center in South India. Indian J Paediatr Dermatol. 2016;17:190–5.
- Pandit VS, Udaya K. A study of neonatal dermatoses in a tertiary care center. Indian J Paediatr Dermatol. 2019;20:36–40.
- Reginatto FP, Muller FM, Peruzzo J, Cestari TF. Epidemiology and predisposing factors for erythema toxicum neonatorum and transient neonatal pustular: a multicenter study. Pediatr Dermatol. 2017;34:422–6.