Common Skin Disorders in childrenpresentation

Skin disorders

1. COMMON SKIN CONDITIONS IN NEONATES

2. Neonatal period: first 4 weeks of extrauterine life NEONATAL SKIN IN COMPARISON WITH ADULT Thinner epidermis Higher epidermal proliferation and desquamation rate Higher transepidermal water loss Thinner, less hairy, weaker intercellular attachment Fewer eccrine and sebaceous gland secretions Increased susceptibility to external irritants Increased susceptibility to microbial infection

3. CLASSIFICATION: Transient Birthmarks Infections Infestations Genodermatosis Neurocutaneous

4. VERNIX CASEOSA White, creamy, naturally occurring biofilm covering the skin of the fetus during the last trimester of pregnancy Composed of degenerated fetal epidermis (Periderm) and sebaceous secretions Very low birth weight infants, i.e., <28 weeks' gestation and < 1000g lack the protective mantle of vernix caseosa At birth, vernix may cover the entire skin surface or only confined to body folds.

5. Function: It performs an epidermal barrier function in utero to facilitate epidermal growth underneath it Acts as an antimicrobial cover, protect against the bacteriologically rich environment of the mother's genital tract Thermal regulation Antioxidant properties by virtue of the presence of antioxidants vitamin-E and melanin in it Its color may reflect intra-uterine problems such as hemolytic disease of newborn, post maturity, where it is of golden yellow color. Fetal distress in utero may stain vernix by bile pigments present in meconium.

6. CUTIS MARMORATA Beni g n cu t a n e ous vasc u lar ph e nomena physiologic se e n in vas o motor neonates a s a n acce n tuated response to cold. Ret ic u late, bluish m ottl i ng of sk i n on tru n k a n d extremities. Usually disappear as the infants is rewarmed. It’s persistence is seen in Downs syndrome, trisomy-18, hypothyrioidism.

7. CUTIS MARMORATA TELANGIECTATICA CONGENITA Congenital, vascular malformation, Present since birth Characterised by fixed patches of mottled skin with a marbled or reticulate blue to pale purple patches Does not disappear after warming (Unlike cutis marmorata) Skin may appear indented due to dermal atrophy Localized or generalized. When localized, lesion tends to remain unilateral, do not cross the midline, and it may be sharply demarcated Limbs are more commonly affected

8. Several congenital abnormalities associated – Craniofacial, Neurological, Vascular, Skeletal, Hypothyroidism Clinical Diagnosis TREATMENT- Most of the lesions improve in the first 2 years of life. Therapy should be deferred to await spontaneous improvement Monitor for development of associated abnormalities

9. SUCKING BLISTER A blister or denuded area seen in neonates, usually seen on the forearm, wrist and fingers Due to vigorous sucking in utero 0.5% of newborn One or two solitary clear bullous lesion with no surrounding erythema. Can also present as an erosion or a crusted lesion Treatment: None, heal rapidly without sequelae Topical antibiotic/Dressing as needed if eroded

10. ACROCYANOSIS (PERIPHERAL CYANOSIS) Functional peripheral vascular disorder characterized by bluish discoloration of skin Caused by vasospasm of the small vessels of the skin in response to cold Usually particularly marked on the palms, soles and around the mouth Absence of cyanosis of warm central parts Resolves with warming of the skin Recurrence unusual after 1 month of age

11. TOXIC ERYTHEMA OF NEWBORN (ERYTHEMA TOXICUM NEONATORUM) Benign, self-limiting disorder of unknown etiology Most commonly, the eruption initially takes the form of a blotchy, macular erythema Most commonly on the trunk, face and proximal parts of the limbs (palms and soles not involved) In more severe cases, urticarial papules arise within the erythematous areas, may be surmounted by small pustules

12. Onset rarely at birth, but in the first few days after birth Usually fade over 3-7 days, but recurrences may occur for upto 6 weeks Systemic symptoms are absent Investigations: Mostly a clinical diagnosis Smear of pustule contents will reveal numerous eosinophils Negative bacterial and viral cultures

13. Histologically the macular erythema shows oedema in the upper dermis, perivascular inflammatory infiltrate comprising principally of eosinophils Papular lesions shows eosinophilic infiltration of the outer root sheath of one or more hair follicles, above the point of entry of the sebaceous duct. Pustular lesions show intrafollicular accumulation of eosinophils immediately below the stratum Corneum An associated blood eosinophilia of up to 20% of the white cell count Treatment: No specific treatment Supportive

14. TRANSIENT NEONATAL PUSTULAR MELANOSIS brown Idiopathi c pustular erupt ion th a t heals with pigmented macules Usually present at birth, more common in black neonates Char a cter i z e d b y 1–3 mm, flac c id, su p er ficial, fragi l e pustules with no surrounding erythema Site – Any site, but predominantly in the chin, forehead, axilla and nape of the neck. Eventually the pustul e s rupture a nd form b r own cr u st and finally a small collarette of scales

15. Pustular lesions usually resolve within 24-48 hours Hyperpigmented macules may persist for about 3 months, sometimes already present at birth Investigation: Smear from pustules content reveals predominance of neutrophils with occasional eosinophils Bacterial culture negative Biopsy shows intra or subcorneal collections of neutrophils with a few eosinophils. Pigmented macules demonstrate basal and supra-basal increase in pigmentation, without any pigmentary incontinence Treatment: No treatment, self resolving

16. ACROPUSTULOSIS OF INFANCY Condition of unknown etiology, characterized by recurrent crops of pruritic, vesiculopustular lesions with a predilection for the palms and soles Onset is usually in the first 3 months of life but lesions may sometimes be present at birth Can also occur on the dorsa of the feet, hands, fingers, ankles, and forearms Lesions appear to start as tiny, red papules, which evolve into vesicles and then pustules over about 24 hr Each crop lasts for 7–14 days, and tend to occur at intervals of 2–4 weeks

17. Healing is succeeded by macular postinflammatory hyperpigmentation. Investigations: Smears from the pustule content show predominance of eosinophils and later neutrophils. Cultures are sterile Histopathology would reveal well-circumscribed subcorneal or intraepidermal aggregations of neutrophils with a sparse lymphohistiocytic infiltrates in the papillary dermis Treatment: Potent topical corticosteroids

18. MILIA Benign, keratinous cysts, which affect about 40-50% of newborn babies Small, firm, pearly- white papules, 1-2 mm in size predominantly occurring on the face of newborn babies Site - cheeks, nose, nasolabial fold and forehead Treatment - Usually disappear spontaneously during first 3-4 weeks. D/D molluscum contageosum – does not usually ap p ea r in i mm e diat e n e on a tal per i od, Sebaceous gland hyperplasia-yellow rather than whitish

19. EPSTEIN PEARLS Yellowish white, keratinous cysts, 1–2 mm diameter, Seen in up to 85% of all neonates Site - Along the alveolar ridges and/or in the midline at the junction of the hard and soft palate Treatment - Generally disappear without treatment within a few weeks

20. SEBACEOUS GLAND HYPERPLASIA Common benign proliferation of the sebaceous glands seen during the first weeks of life Result from maternal androgenic stimulation of sebaceous gland Multiple, uniform, pinpoint, yellowish papules 1–3 mm in diameter most prominent on the nose, cheeks, upper lip and forehead Treatment - Resolves within few weeks

21. MILIA R IA Miliaria is a disorder due to blockage of eccrine sweat ducts Subdivided into three subtypes depending on the level of blockage: miliaria crystallina (stratum corneum), miliaria rubra (mid‐epidermal) and miliaria profunda (dermal– epidermal junction) 3–8% of neonates Predisposing factors - Immature sweat ducts, Occlusive clothing, high heat and humidity

22. Miliaria crystallina: Presents as crops of clear, thin‐walled, superficial vesicles 1–2 mm in diameter, without associated erythema. Generally rupture within 24 h, and are followed by bran- like desquamation Presence of intracorneal or subcorneal vesicles in communication with the sweat ducts Most frequently during the first 2 weeks of life, and are particularly likely to be seen on the forehead, scalp, neck and upper trunk

23. Miliaria rubra (prickly heat): Erythematous papules and papulovesicles about 1–4 mm in diameter, on a background of macular erythema Usually begin after second week of life and predominate in the trunk and intertriginous areas where occlusion by clothing is accentuated Lesions can be itchy or sore, child may be restless and distressed Miliaria profunda is very uncommon in neonates as it usually occurs in adults where there have been repeated episodes of miliaria rubra.

24. Investigations: Clinical diagnosis Smear of a vesicle/pustule will show an absence of eosinophils Treatment: Light clothing, cool bath and avoidance of heavy blankets Miliaria crystallina improves spontaneously as the sweat ducts mature Milaria rubra improves if the predisposing aetiological factors (high heat/humidity and occlusion) are removed Antibiotics may be needed if staphylococcal infection occurs, but this is rare.

25. BIRTHMARKS (CONGENITAL NAEVI) Birthmarks are congenital, benign irregularity on the skin which is present at birth or appears shortly after birth Represent an excess of one or more of the normal components of skin per unit area: blood vessels, lymph vessel, pigment cells Vascular birthmarks are most common.

26. SALMON PATCH (NEVUS SIMPLEX) Most common vascular birthmark of infancy. Seen in 40% of all newborns Cause: Area of superficially dilated capillaries Appears as irregular dull, pinkish – red macules with poorly defined borders Site - On the face (angel kiss), nape of neck (stork bite) Become more intense in colour when child is crying Most of these lesions spontaneously disappear within a year.

27. PORT WINE STAIN (NAEVUS FLAMMEUS) Vascular birthmark, about 0.3% of newborns Large, irregular, deep red or purple macule with well defined borders Usually unilateral, often on the face. Represents a vascular malformation involving mature capillaries. Lesions do not enlarge but persist throughout life Treatment: Pulsed dye laser Cosmetic masking, excision, grafting

28. STRAWBERRY MARK (CAPILLARY HAEMANGIOMA) Localised superficial haemangioma U s ual l y not pres e nt a t birth, d e ve lops durin g f i rst f e w weeks of life. Present in about 10% of infants Most of them start off as small red macules and usually grow rapidly during the first four or five months of life Later appear as circumscribed oval or round, soft domed swelling of intense scarlet-red color. Site – Head, neck region and trunk Over 90 percent disappear by the age of 7 years Treatment - Vascular specific Pulsed dye laser

29. MONGOLIAN SPOTS Blue - gray, poorly cir c umscribed, single or mult i ple, macular lesion of various sizes Entrapment of melanocytes in dermis of developing embryo, the cells fail to reach their proper location in the epidermis Usually present at birth or appears within the first weeks of life Most commonly over lumbosacral region Common in asian, black and hispanic infants Most fade during first two years of life Persist – Q switch laser, bleaching creams

30. CONGENITAL MELANOCYTIC NEVI (CMN) Benign proliferations of cutaneous melanocytes that arise as a result of abnormal growth, development, or migration of melanoblasts Affecting approximately 1% of newborns Present at birth or become apparent within the first year of life Round or oval with smooth, well-defined borders, and the surface texture can be papular, verrucous, or cerebriform Initially a nevus may be light in color, flat, or hairless, it can become more pigmented, raised, and acquire long, coarse hairs

31. Lesions classified according to size - small <1.5cm, medium 1.5cm-20cm, large >20cm in greatest diametrer or covering >2% BSA. Lifetime risk for malignant transformation depends largely on size, from 0-5% for small CMN, and 5-10% for large CMN 70% of melanomas occur within the first decade of life Naevi over the cr a nium or spine h a v e associat i on wit h Neurocutaneous melanosis Treatment: Early treatment with full-thickness excision followed by grafting, otherwise close observation Dermabrasion, chemical peels, and lasers improve cosmetic appearance

32. NEVUS OF OTA Extensive, bluish, patchy, unilateral dermal melanocytosis that affects the sclera and the skin adjacent to the eye, distributed along the first and the second branches of the trigeminal nerve More common in Asians (0.014-0.034%) and blacks Most are present at birth or develop during first year of life Nevus of ITO – Same features as OTA, only distinguished by location in the acromioclavicular region and the upper chest. Treatment - Q‐switched lasers, Cosmetic camouflage

33. BLUEBERRY MUFFIN BABY Widespread, purple, erythematous, oval or circular macules, papules and nodules reflecting dermal erythropoiesis May be frank petechiae on the surface of some of the lesions Generalized distribution of 1 to 7 mm purpuric papules, especially on head, neck, and trunk Generally fade into light brown macules within a few weeks of birth This type of lesion has been recorded in a number of congenital infections (Rubella, cytomegalovirus, Coxsackie B2, syphilis, toxoplasmosis ) and a variety of disorders (Rhesus haemolytic anaemia, ABO incompatibility, Neonatal lupus erythematosus etc)

34. APLASIA CUTIS CONGENITA A rare disorder characterized by a focal absence of epidermis, dermis, and in some cases subcutaneous tissues – including bone and dura mater Most presentations (80–90%) involve the vertex of the scalp Estimated rate of incidences is 1-3 in 10,000 births Oval, sharply marginated, depressed, hairless area covered by wrinkled epithelial membrane, or may appear as ulcer which heals with scar formation. A ring of hair around the defect, known as a ‘hair‐collar’ sign, is thought to be associated with an increased risk of underlying defects

35. Can occur in isolation or association with other types of disorders (limb abnormalities, epidermal nevi, embryologic malformations, trisomy 13, epidermolysis bullosa etc) Treatment: Small defect- Surgical excision with mobilization of scalp and closer. Large defect- Hair transplantation Management of associated disorders accordingly, if present

36. NEONATAL ACNE Prepubertal acne can be divided into five subgroups: neonatal, infantile, midchildhood, preadolescent and adolescent Thought to be due to androgens (maternal & infant) May affect up to 20% of neonates, more common in boys Presents at or shortly after birth with erythematous papulopustular lesions, and comedones Site - commonly on cheeks, chin and forehead

37. T re a tme n t : Usually settles spontaneously and leaves little scarring. Gentle cleaners, oil‐free emollients If marked pustules - topical azole cream

38. NEONATAL CEPHALIC PUSTULOSIS Historically referred wrongly as neonatal acne because of their clinical similarity Presents in the first 3 weeks of life Prevalence varies between 10 and 66% of newborns Postulated to develop in association with Malassezia Characterized by erythematous papulopustules, surrounded by erythematous halo Site - cheeks, chin, eyelids, neck and upper chest

39. Absence of comedones and presence of pustules surrounded by erythematous halo help to differentiate this entity from neonatal acne Investigation: Isolation of M. furfur in pustule content (50-60%) Treatment: Self-limiting nature, heals in 1-3 months without scarring Persist/widespread – 2% ketoconazole cream for 15 days

40. INTERTRIGO Superficial inflammatory dermatosis involving body folds that develops through friction Heat, mo i s ture, frict i on a n d sweat re t ent i on ind u ce maceration and inflammation. Seco n daril y infected b y b a cteri a l (s . a u reus, group A streptococci) or fungal (candida) infection.

41. Initially skin is red and slightly macerated, when separated show erythema of contagious surface. Itching, burning and odour are common symptom. TREATMENT open wet compresses Dusting powder Antibiotics –cephalexin 40-50mg/kg/day or cloxacillin50-100mg/kg/day for 10 days or fungicidal nystatin cream 4-5 times /day for 3-4 days may be used.

42. DIAPER DERMATITIS (NAPKIN DERMATITIS) Acute inflammatory reaction of the skin associated with the wearing of napkins. Irritant contact dermatitis – due to occlusive contact of urine and faeces with skin. Rash is usually bounded by the margins of the nappy with sparing of the inguinal fold. After prolonged contact, a papuloerosive eruption occurs with formation of multiple small ulcers, called Jacquet’s ulcers. Secondary infection by candida is common - the erythema gets worse and there are satellite papular-pustulous lesions

43. Investigations: Skin swabs may be useful to confirm yeast or bacterial infection. Management: Remove the contactants, keep the diaper area dry . Frequent diaper change. Contamination by Urine or Feces should be rinsed gently with warm water. Zinc cream or petroleum jelly is useful Mild topical steroid such as 1% hydrocortisone cream on inflamed skin once or twice daily for 1–2 weeks Topical antifungal if secondary infection present e.g. ketoconazole, nystatin at 100,000U/g or 1% miconazole nitrate Jacquet’s ulcers Candidosis

44. S E B O RR H E I C D E R M A TITI S (Cr ad l e C a p ) Characterized by large flakes of yellowish scale on the scalp, may become matted into large plaques of crust. Etiology - unknown Sit e - Scalp , face, postauricula r , prest e rn a l a n d intertriginous areas etc. Begins wit h a no n-e c zemato u s, eryth e ma t ou s , scaly dermatitis of the scalp and spread downward Treatment: Topical weak corticosteroid - 1% hydrocortisone Mild baby shampoo, ketoconazole 2% shampoo

45. NEONATAL LUPUS ERYTHEMATOSUS Occurs in about 1-2% of babies born to mothers with clinical or subclinical autoimmune connective tissue disease (primarily SLE and Sjögren's syndrome) Transplacental passage of maternal autoantibodies ssA- Ro and ssB-La is thought to play role in pathogenesis Babies from subsequent pregnancies have a 20–25% risk of skin or cardiac disease Present with either skin lesions (90%) or cardiac lesions (1%) Well‐defined areas of macular or slightly elevated erythema, frequently annular

46. Site - face, particularly the forehead, temples and upper cheeks ‘Spectacle‐like’ distribution of lesions around the eyes is especially characteristic Congenital heart block (typically begins during the 2nd or 3rd trimester) Associated - hepatosplenomegaly, anemia, leukopenia, thrombocytopenia, and/or lymphadenopathy Investigation- skin biopsy, circulating autoantibodies in both the mother and the child, ultrasound or electrocardiography Treatment- Sunprotection Except for cardiac involvement, usually resolves in 6-12 months 50% with cardiac involvement will require a pacemaker

47. COLD PANNICULITIS Results from cold exposure to adipose tissue Fat of newborn are more highly saturated than that of older children, with the effect that it solidifies at a higher temperature Indurated, warm, red, subcutaneous plaques and nodules appear within hours or days of appropriate cold exposure Skin biopsy shows a lymphohistiocytic infiltrate around blood vessels at the junction of dermis and subcutaneous fat Induration resolves over a period of a week often leaving some residual postinflammatory hyperpigmentation Self-limiting disorder and requires only symptomatic relief

48. CONGENITAL SYPHILS Transplacental infection by Treponema pallidum Lesions usually appear between 3-8 weeks of age 15% of children born to untreated mothers are infected Lesions are reddish brown in colour, may be macular or papular Sites of predilection are the ano‐genital area, the face and the palms and soles If ulcerative in nature, they are highly contagious Syphilis rhinitis (snuffles) most important and frequent sign

49. Other features include bone abnormalities, anemia, thrombocytopenia, fever, hepatosplenomegaly, lymphdenopathy, and poor feeding Investigations- X‐ray of long bones CSF examination Dark‐field microscopy and/or PCR from exudates Treatment- Aqueous crystalline penicillin 50,000 units/kg/dose IV every 12 h during the first 7 days of life and every 8 h thereafter for a total of 10 days

50. NEONATAL HERPES SIMPLEX Incidence about 7/100 000 live births Mostly from transmission of HSV type 1 (20%) and HSV type 2 (80%) through the contact with an infected genital tract during delivery. Intrauterine 5% Common in premature and low birth weight Of infected babies, 70% develop the skin/mucosal lesion, 90% systemic Lesions appear between day 2 and 20 Clustered red papules and vesicles, then become pustular, crusted and erosion over the following 2-3 days

51. HSV infection in newborns usually develops in one of three patterns: Localized to the skin, eyes, and mouth (40%) Central nervous system (CNS) disease (35%) Disseminated disease involving multiple organs (25%) Investigations: Serology, Viral culture, PCR Treatment: Skin & mouth disease - Acyclovir 20mg/kg-8hrly IV for 14 days Encephalitis & systemic disease -21 days.

52. NEONATAL VARICELLA Neonatal varicella is mostly caused by maternal chickenpox acquired during pregnancy (25% of cases)

53. FETAL VARICELLA SYNDROME

54. Cutaneous lesions: Lesions are polymorphous- papules, pustules and vesicles The infant's rash usually occurs toward the end of the 1st week to the early part of the 2nd week of life Vesicles usually develop over 1st 3-10 days of life Localized scarring, most common cutaneous feature of FVS Localized absence of skin, usually on a limb Investigations: PCR, Serology (IgM and IgG)

55. Treatment- Newborns whose mothers demonstrate varicella 5 days before to 2 days after delivery should receive 1 vial of VZIG as soon as possible. All premature infants born <28 wk gestation to a mother with active varicella at delivery (even if the maternal rash has been present for >1 wk) should receive VZIG The infant should be treated with acyclovir (10 mg/kg every 8 hr IV) when lesions develop

56. CONGENITAL RUBELLA congenital rubella syndrome (85%) 0-16 weeks After 16 weeks normal development, slight risk of deafness and retinopathy Congenital rubella typically results from maternal infection Preconception minimal risk 0-12 weeks 100% risk of fetus being congenitally infected resulting in major congenital abnormalities. Spontaneous abortion in 20% of cases.

57. Congenital Rubella Syndrome Classical triad consists of cataracts, heart defects, and sensorineural deafness. Many other abnormalities, divided into transient, permanent and developmental. Transient low birth weight, hepatosplenomegaly, thrombocytopenic purpura bone lesions, meningoencephalitis, hepatitis, haemolytic anemia pneumonitis, lymphadenopathy Permanent Sensorineural deafness, Heart Defects (peripheral pulmonary stenosis, pulmonary valvular stenosis, patent ductus arteriosus, ventricular septal defect), Eye Defects (retinopathy, cataract, glaucoma, myopia) Other Defects (microcephaly, diabetes mellitis, thyroid disorders) Developmental Sensorineural deafness, Mental retardation, Diabetes Mellitus, thyroid disorder

58. Discrete rounded, red or purple infiltrated macules, 3-8 mm in diameter Site – face, scalp, back of neck and trunk Blueberry muffin lesions Other skin manifestation – cutis mamorata, seborhoea and hyperpigmentation of the forehead, cheeks, umbilical area and discrete deep dimples over bony prominences, particularly the patellae Investigations: PCR - Rubella virus isolation Rising titres of antibody (mainly IgG) - EIA Treatment: Supportive care

59. HAND, FOOT & MOUTH DISEASE Caused by Coxsackie A -1 6 virus (Occasionally A5-A10) Symptoms can develop within 2 weeks after birth. Abrupt onset scattered papules that progress to oval or linear vesicle. Preceded by Fever Distribution- Palms, fingertips, interdigital webs soles & buccal mucosa. Clears spontaneously in about 7 days . Treatment- Rest, fluids, Acetaminophen

60. BULLOUS IMPETIGO A contagious infection of the superficial epidermis Most often caused by phage group II strains of staphylococcus aureus Due to staphylococcal exfoliative toxins (exfoliatin A–D), which target desmoglein 1 Lesions do not generally appear until the second week of life Superficial flaccid blister without erythema, initially clear, yellow fluid that turns cloudy and dark yellow Rupture easily, dries and form a honey – colored crust Associated systemic symptoms - fever, malaise, generalized weakness, and diarrhea

61. Investigations: Swab culture – bacteria Blood count may reveal neutrophil leucocytosis Biopsy - Acantholytic cells, Cleavage plane is subcorneal or upper granular layer, Minimal inflammatory infiltrate in upper dermis Treatment: Normal saline or potassium permanganate soaks to remove the crust. Topical antiseptic (povidone iodine, hydrogen peroxide cream, chlorhexidine) or topical antibiotic (fusidic acid, mupirocin). Systemic antibiotics in extensive lesions

62. STAPHYLOCOCCAL SCALDED SKIN SYNDROME (SSSS) ( Ritter’s disease) Syndrome of acute exfoliation of the skin caused by epidermolytic toxin A and/or B (exotoxin) produced by staphylococcus aureus The toxins act at the zona granulosa of the epidermis, causing cleavage of desmoglein 1 complex Greater incidence of this condition in neonates due to less efficient metabolism and excretion of the toxin. These toxins reach the skin via the circulation from a distant focus of infection, usually in the umbilicus, breast, conjunctiva or site of circumcision or herniorrhaphy

63. Start as a macular, orange‐red, scarlatiniform eruption Lesion generally becomes more extensive, and over the next 24–48 h turns to a more confluent, deep erythema with oedema Surface then becomes wrinkled before starting to separate, leaving raw, red erosions. First appear on face, axillae and groins then spread all over body. Extreme tenderness of the skin is an early feature Child is pyrexial and distressed

64. Investigations: Nikolsky’s signs - positive. Tzanck smear Skin biopsy, which shows intraepidermal cleavage at the granular layer Bacterial culture from skin, blood. The intact bullae are sterile Treatment: Intravenous penicillinase-resistant penicillin, such as nafcillin or oxacillin Other antibiotics include cephalosporin and clindamycin Vancomycin should be considered in areas with a high prevalence of Community Aquired-MRSA Fluid and electrolyte balance.

65. CANDIDIASIS Early weeks after birth, in the form of oral candidiasis. Acquired during delivery from the mother’s genital tract (frequency of vaginal candidiasis 20% - 25%) Rash is usually focused in the perianal area, and is a deep ‘beefy’ red colour, with a moist appearance, often with pustules at the periphery Pseudomembranous form is the most common and appears as white plaques on the buccal mucosa, palate, tongue, or the oropharynx Painful inflammation of tongue, soft and hard palate, buccal & gingival mucosa can extend to esophagus/ pharynx.

66. Congenital Candidiasis : A rare condition seen at birth, due to ascending infection from the genital tract. Candida is able to find its way into the amniotic fluid without prior rupture of membranes Two forms have been described: Congenital cutaneous candidiasis: presents within 12 hours of birth. A macular erythema that may evolve into a papular, vesicular or pustular phase over a period of 1–3 days, finally results in extensive desquamation. Palmar and plantar pustules are regarded as hallmark Congenital systemic candidiasis: An invasive infection with a high mortality rate, especially in VLBW infants. At least 50% do not have a cutaneous rash. Presenting signs are pneumonia (most common), meningitis, candiduria and/or candidemia.

67. Investigations: Skin/mucosa swab and scraping - candida Endoscopy in extensive oral candidiasis, to see the extent of the lesions Treatment: Clotrimazole 1% used as mouth paint 4 times a day Nystatin, amphotericin B or miconazole gel applied several time a day. Systemic antifungal therapy for systemic infection (Amphotericin B drug of choice - amphotericin B deoxycholate 0.5–1.0 mg/kg once daily) Maintain proper oral hygiene

68. SCABIES Scabies may present as early as three to four weeks of age but is never present at birth Neonates do not itch, but present with extensive rashes, irritability, poor feeding, and failure to thrive. Vesicles are common, leading to early pustulation, crusting, and scaling. Eruption is generalized, including involvement of the head, neck, face, palms, and soles, with an early tendency for pustule formation

69. Investigations: Scraping of skin from an unscratched burrow- microscopy revealed female mite &/or her eggs. Treatment: Permethrin 5 % cream at bedtime to all skin surfaces in neonate and from the neck down in older family members Disinfection of recently used clothing and linens

70. NEONATAL PURPURA FULMINANS Potentially lethal disorder characterized by progressive haemorrhagic necrosis of the skin due to abnormal coagulation and microvascular occlusion Usually due homozygous deficiency of protein C or, less frequently, protein S Rarely, can occur due to infection, such as group B Streptococcus , methicillin‐resistant Staphylococcus aureus Lesions most characteristically appear within first 12 h of life Symmetrical and well‐defined ‘lakes’ of confluent ecchymosis Patient is frequently febrile

71. Onset is sudden, and the lesions enlarge rapidly, with coalescence and the development of haemorrhagic bullae and central necrosis. There is surrounding erythema and the lesions are tender Site - Most often on the limbs, particularly at sites of pressure Investigations: Thrombocytopenia, Polonged aPTT and PT Bacterial culture Histopath – cutaneous necrosis without evidence of inflammation, and occlusion of blood vessels with platelet-fibrin thrombi Treatment: Initially, fresh frozen plasma 10–15 mL/kg/12 h If protein C deficiency is confirmed, onward therapy with protein C concentrate should continue until the skin lesions have healed Longer term treatment is with oral anticoagulants Any concomitant infection needs to be treated as well

72. ICHTHYOSIS Ichthyosis - excessive scaling of skin. Major hereditary types Vulgaris, Recessive X-linked, Autosomal recessive, Keratinopathic Autosomal recessive congenital ichthyosis Includes all non‐syndromic autosomal recessive congenital forms of ichthyosis without a tendency toward blistering Includes harlequin ichthyosis, bathing suit ichthyosis, lamellar ichthyosis, self‐improving congenital ichthyosis, Colloidon baby etc

73. Harlequin ichthyosis Most devastating type of ARCI. Often lethal in around 44% case Mutations in the ABCA12 gene Neonates are born with armour‐like skin (truncal plates with fissuring) Impaired movement and the ability to drink and breath More prone to infection of the skin, as well as other organs such as the lungs (major cause of death) Biopsy - enormous thickening of stratum corneum. Parakeratosis and hypergranulosis and reduced non‐polar lipids Electron microscopy – numerous abnormal lamellar bodies in stratum granulosum

74. Collodion baby Most autosomal recessive congenital ichthyosis (ARCI) present at birth as collodion babies Baby encased in shiny parchement-like membrane which may impair respiration and sucking . Usually peels off within the first 4 weeks of life. In 80% of cases, collodion baby is followed by the onset of an ARCI subtype Around 10–20% develop into self‐improving congenital ichthyosis

75. TREATMENT: High humidity incubator (60-80%) with close monitoring of body temperature. Bland ointments two to four times a day In severe disease, the oral retinoids such as acitretin, isotretinoin at low dosage, up to 0.5 mg/kg body weight

76. NEUROFIBROMATOSIS 1 (von Recklinghausen's disease) Autosomal dominant disease Due to mutation of NF1 gene in chromosome 17 NF1 gene – neurofibronin (tumour suppressor) Most common type of Neurofibromatosis (90%), with a prevalence of about 1 in 3,000 births Present at birth or shortly after Characterised by the presence of: 6 or more caf é-a u - lait spots (d e f i ned oval - s h aped light brown patches greater than 0.5cm in diameter) Multiple neurofibromas Freckling (under the armpits and areas of skin folds) Lisch nodules (tiny tumours on the iris of the eye)

77. INCONTINENTIA PIGMENTI X-linked dominant multisystem disease that is usually lethal in males The skin lesions develop in four stages: Erythematous papules and vesicles appear in crops in linear streaks along the lines of Blaschko, usually at birth or within the first few weeks of life, each crop lasting one to two weeks The verrucous stage follows, consisting of hyperkeratotic warty papules or plaques in linear or swirling patterns The third pigmented stage presents as streaks of hyperpigmentation in a swirled pattern The hyperpigmented streaks then may evolve into a final stage of hypopigmentation and atrophic patches or streaks.

78. Associated Systemic abnormalities occur in nearly 80 percent of patients Teeth 80% - delay in eruption, unusually shaped, typically pegged or cone-shaped Nails 40% - nails may be ridged, pitted, thickened or completely disfigured Hair 50% - eyebrows and eyelashes, may be coarse, wiry and lack lustre Eyes 20-35% - Abnormal blood vessel growth resulting in scarring, blindness Central nervous system 30% - seizures Treatment Prevent secondary bacterial infection of skin lesions Topical tacrolimus and topical corticosteroids has been reported to hasten the resolution of the inflammatory stage.

79. T H AN K Y O U