Neonatal Dermatology: Part I

1. Neonatal Dermatology:Part I Mikelle Key-Solle MD 8/05

2. Objectives Review the appearance of common benign dermatological findings in neonates Describe the assessment and management of less common but significant dermatological disorders in neonates Provide a framework for classifying and identifying neonatal skin findings

3. Part 1 What�s the common link?

5. Desquamation Normal when develops 24-48 hours after birth and abates up to 2 weeks If present at birth, then consider postmaturity or icthyotic conditions

6. These infants were born with this shiny membrane covering the entire body.

7. Icthyosis Congenita AKA Collodion baby Infant covered by thick, taut, shiny, red, translucent membrane that cracks with inspiration, then sheds in large sheets May have �O mouth� deformity Complications include significant insensible water losses with hypernatremia, hypothermia, infection, aspiration of shedding squamous tissue May evolve to icthyosis, Gaucher disease, or no skin disease Supportive treatment with careful fluid management, high humidity incubator to minimize water loss, wound care with non-occlusive lubrication

9. Harlequin Fetus Rare keratinizing disorder with thickened ridged, cracked skin forming plates over the entire body at birth Disfigures facial features and constricts digits Hair and nails may be absent Respiratory difficulty, poor suck, usually death in days Rare survivors have severe ichthyosis and neurologic impairment

11. Contact Dermatitis AKA diaper dermatitis, irritant dermatitis Erythema and scale in response to chemical irritation (urine/feces/soaps/topical preparations); betadine used at time of circumcision for our example Patchy or confluent, sparing skin folds Tx with frequent diaper changes, gentle cleansing with water qod, barrier creams, air drying If persistent, light application of 0.5-1% topical hydrocortisone cream after each diaper change If severe with vesicles/bullae/fissures/erosions, add Burow�s solution to regimen

13. Candidal Diaper Dermatitis Intensely erythematous, confluent plaque with scalloped/sharply demarcated border Satellite pustules are hallmark Contact, seborrheic, atopic dermatitis serves as portal of entry Erosive balanitis in males Tx with anti-fungal ointment (nystatin, clotrimazole) qid until rash cleared for 2 days; zinc oxide paste or thick barrier cream Consider short course of topical hydrocortisone if inflammation severe Tendency to recur due to GI colonization

15. Seborrheic Dermatitis AKA Cradle cap Chronic inflammation of scalp, face, intertriginous areas Greasy yellow crusting with underlying erythema resulting from overproduction of sebum Treat with lubrication, anti-seborrheic shampoo, and gentle scraping Differentiate from psoriasis, eczema, contact dermatitis, candidiasis

17. Psoriasis Erythematous papules coalescing to plaques, sharply demarcated irregular borders, thick silvery yellow/white scale Auspitz sign--pinpoint bleeding with removal of scale Koebner response--new lesions at sites of trauma Scalp, elbows, umbilicus, diaper areas common May mimic seborrhea, eczema, Candidosis, or perianal strep Tx with tar preparations, UV light, topical corticosteroids, keratinolytic agents

19. Langerhan Cell Histiocytosis AKA Letterer-Siwe syndrome, Histiocytosis X X-linked proliferation of histiocytes in viscera/bones Progressive diaper dermatitis initially resembling seborrheic dermatitis, then becoming hemorrhagic and erosive; similar rash on scalp/trunk/limbs Can similarly infiltrate multiple organ systems with organ dysfunction and possibly death; eosinophilic granulomas of bone common Hallmark is Langerhan cell Birbeck granule seen on electron microscopy (tennis racket-shaped intracellular bilamellar granule)

21. Congenital Syphilis Results from transplacental transmission of spirochetes during primary, secondary, or early latent syphilis Risk factors include inadequate prenatal care and cocaine abuse Fetal/perinatal death in up to 40% Early signs (first 2 years of life): multi-organ system involvement Mucocutaneous, erythematous, maculopapular to vesiculobullous eruption present at birth, which desquamates on hands/feet Coombs negative hemolytic anemia, thrombocytopenia, HSM CNS and ocular abnormalities (chorioretinitis) Osteochondritis of joints, periostitis of long bones/skull produces irritability +/- decreased movement of limbs

22. Part 1 What�s the common link?

23. Part 1: Papulosquamous Eruptions

24. Neonatal Dermatology:Part 2 What�s the common link?

26. Acne neonatorum Hypertrophic sebaceous glands causing closed comedones on cheeks, forehead, and face in first month of life; occasional open comedones Unknown etiology, but thought secondary to maternal androgen stimulation Associated with maternal use of hydantoin and lithium Resolves over months without treatment

28. Sebaceous Gland Hyperplasia Profuse superficial yellow-white 1-2 mm papules over cheeks and nose representative of hyperplastic glands Often superficial and can be wiped off Can take months to resolve

30. Milia 1-2 mm, firm inclusion/retention cysts most commonly on nose/chin Filled with keratinaceous material in the pilosebaceous follicle Up to 50% of neonates Cannot be wiped off and not associated with sweat retention

32. Epstein�s Pearls AKA Bohn�s nodules Associated with milia and represent similar keratinaceous material-filled inclusion cysts Resolve without treatment or symptoms

34. Miliaria Crystallina Sudden eruption of pinpoint clear vesicles over large body surface area Occluded eccrine sweat ducts by keratinaceous plug in duct Noninflammatory, asymptomatic, superficial Associated with hot/humid weather, fever, or excessive bundling Secondary to the relative immaturity and delayed patency of sweat duct Often desquamation upon healing

36. Miliaria Rubra Erythematous, papulovesicular, extrafollicular eruption deeper than crystallina Localized to site of occlusion or flexural areas (neck, groin, axillae) subject to friction May become eroded/macerated, so confused with candidosis and folliculitis Swelling of ductal epidermal cells?duct obstruction?keratinaceous plug and surrounding erythema Responds to cooling/defervescence Repeated episodes may lead to miliaria profunda, with deep rupture of sweat gland and disturbance of heat regulation

37. Miliaria Profunda

39. Erythema Toxicum AKA Urticaria Neonatorum Benign, transient 2-3 cm erythematous maculopapular rash scattered over trunk/face/limbs sparing palms and soles Affects about 50% of infants May have central papule/vesicle/pustule First appears 24-48 hrs of life, last 2-3 weeks Eosinophils on Wright�s stain

41. Transient Neonatal Pustular Melanosis Three phases: Early evanescent superficial pustules 2-3 d Pustules rupture with collarette of fine scale Late hyperpigmentation resolving by 3 months Occurs on neck, forehead, lower back followed by trunk No bacteria or eosinophils on smear

43. Sucking Blisters Superficial bullae on upper limbs/hands present at birth Diagnose by observing infant sucking area

45. Neonatal/Perinatal Herpes Simplex Active primary maternal infection at delivery has 33-50% infant attack rate, but only 25% of mothers have symptoms 40% of infections occur during first week of life 3 major catagories of disease Localized skin, eye, mouth: avg 5-6 days of life, 70% progress if untreated CNS infections: avg 8-12 days of life Disseminated infection: avg 5-6 days of life Typical grouped vesicular ulcerative lesions only present in 30-40% Tzanck smear of unroofed lesion, conjunctival/nasal/oral/rectal/skin swabs, DNA PCR on CSF may all aid in diagnosis Tx with IV acyclovir +/- cephalosporin for bacterial superinfection

47. Neonatal Varicella Crops of intensely pruritic erythematous macules evolving to papular then vesicular lesions Vesicles become cloudy, umbilicate, then rupture with crusting Particularly high mortality when contracted less than 1 week prior to delivery due to inadequate maternal antibody transfer May present with prodrome of fever, irritability, abd pain, anorexia 24-48 hours before rash Complications include pneumonitis, encephalitis, hepatitis, corneal ulceration Tx with VZIG if maternal symptoms develop 5 days prior thru 2 days after delivery +/- IV acyclovir if infant has severe symptoms Congenital infection during 1-2nd trimester characterized by cicatricial skin lesions, microcephaly, microphthalmia, cataracts, and shortened malformed limbs

49. Infantile Acropustulosis Cyclic crops of intensely pruritic papules evolving into vesiculopustular lesions within 24 hours Located on palms, soles, sides of feet Lesions then crust over and resolve in 1-2 weeks Cycles every 3-4 weeks for up to 2 years PMNs +/- eosinophils on smear Differentiate from scabies Treat with topical steroids +/- oral antihistamines for itching

51. Congenital Cutaneous Candidosis Rare condition with vesiculopustular lesions anywhere on body in otherwise well infant at birth or within 12 hours of birth Presumed ascending infection from heavily colonized mother May become disseminated with intermittently positive blood/CSF cultures CSF cx positive in one third Treat with topical antifungal; ampho B for disseminated disease

53. Staphylococcal Pustulosis AKA bullous impetigo, Staph scalded skin Disease ranging from localized bullae to generalized cutaneous involvement with systemic illness/fever/irritability Sterile bullae rupture 2-5 days later with crusting Tender erythroderma of flexural/peri-orificial areas with denudation in large sheets (+Nikolsky sign) indicative of scalded skin syndrome Mediated by hematogenous spread of exfoliative/epidermolytic toxins secondary to decreased renal clearance Treat with penicillin +/- clindamycin for decreased toxin production

55. Epidermolysis Bullosa Heterogeneous group of congenital, hereditary blistering disorders, but all characterized by blisters induced by trauma and exacerbated by warm weather Blisters present at birth in most forms with + Nikolsky sign Treatment primarily wound care, monitoring for superinfection, genetic counseling Prognosis extremely variable depending on type

57. Aplasia Cutis Congenita Congenital absence of skin layers Most often on scalp but can occur anywhere Usually isolated anomaly, but can be associated with syndromes/anomalies If found with blisters/nail dystrophy, consider EB Complications include hemorrhage, local infection, meningitis

59. Mastocytosis Spectrum of disorders from solitary cutaneous nodules to diffuse cutaneous/systemic involvement Urticaria pigmentosa is most common form Crops of lesions, usually in kids <2 years Dermatographism of normal intervening skin common Characterized by dermal aggregates of mast cells Intense pruritis, + Darier sign, often blisters which become hyperpigmented orange-peel plaques with pebbly appearance Systemic involvement characterized by intermittent flushing, tachycardia, hypotension, wheezing, syncope, diarrhea (histamine release) Most spontaneously involute by adulthood

61. Incontinentia Pigmenti AKA Bloch-Sulzberger Disease X-linked dominant syndrome with four phases: Erythematous linear streaks/plaques of vesicles on limbs and circumferentially around trunk; birth to 4 months (pictured on left); eosinophils predominant in vesicles Verrucous plaques on limbs Pigmented whorls following Blaschko lines (pictured below) Hypopigmented, hairless, anhidrotic streaks Associated with alopecia/wiry hair, dental abnormalities, MR, CNS and ocular abnormalities, dystrophic nails Lethal in male embryo, so only expressed by heterozygous females Presentation at birth may be in any of first three phases; not all phases apparent in a given patient

62. Lines of Blaschko

63. Part 2 What�s the common link?

64. Part 2: Vesiculopustular Eruptions

65. Neonatal Dermatology:Part 3 What�s the common link?

67. Nevus Vasculosis AKA Common Hemangioma, Strawberry Nevus Benign, bright red to red-blue vascular malformations Congenital present at birth and usually rapidly involute in first year Infantile appears first few weeks of life and usually grow, stabilize around 9 months, then involute (50% at 5y, 70% at 7y, 90% at 9y) Refer if affects vital structures Eyelid?astigmatism and amblyopia �Beard� distribution assd w/ upper airway lesions?stridor Fingers, eyes, lips, nasal tip, ears or face assd w/ psychological distress Midline back assd w/ spinal dysraphism (tethered cord) Numerous hemangiomas may indicate visceral involvement, which can cause high output heart failure or Kasabach-Merritt syndrome (consumptive coagulopathy) if cavernous If tx needed, oral/intralesional steroids, interferon, vincristine, laser, excision

69. Subcutaneous Fat Necrosis Inflammatory disorder of adipose tissue that occurs in first 4 weeks of life May have history of birth asphyxia or difficult labor Asymptomatic, rubbery, erythematous to violaceous plaques/nodules on cheeks, buttocks, back, thighs, upper arms Resolve without scarring in weeks to months, but may have calcium deposits First 6 months, monitor for signs of hypercalcemia (unknown mechanism) including lethargy, poor feeding, FTT, irritability, vomiting, ARF, short QT

71. Dermal Erythropoiesis AKA Blueberry muffin rash Red to blue-red diffuse nodules Ddx extensive: Intrauterine viral infections, esp CMV, rubella Intrauterine toxo Congenital leukemia Rh hemolytic disease

73. Nevus Sebaceous AKA Nevus sebaceous of Jadassohn Yellow-orange plaque on head/neck without hair Becomes verrucous and nodular during teens with 10-15% risk of malignant transformation Complete excision prior to teens recommended

75. Juvenile Xanthogranuloma Firm, dome-shaped, yellow/pink/orange papules/nodules up to 4 cm diameter usually present at birth 10 times more common in white infants Scalp, face, upper trunk most common Most regress spontaneously during first few years with residual pigmentation and atrophy Monitor for intraocular infiltration

77. Congenital Melanocytic Nevus Hyperpigmented macules affecting 1% of infants Classified as small <2 cm, intermediate 2-20 cm, giant >20 cm Increased risk malignant melanoma if on head/midline or giant 50% of melanomas which arise from giant nevi do so by 5y with around 50% mortality

79. Polythelia AKA Accessory Nipples Brown macule located along the milk line, often confused with congenital nevus Usually darken at puberty/pregnancy Associated with urinary and cardiovascular anomalies No treatment necessary

81. Epidermal Nevus Hamartomatous lesions with focal hyperplasia of epidermis/adnexal structures often visible at birth May be discolored, slightly scaly patch that becomes more linear, thickened, verrucous, and hyperpigmented May resemble verrucous stage of incontinentia pigmenti or nevus sebaceous

83. Smooth Muscle Hamartoma Focal hyperplasia of smooth muscle associated with arrector pili in hair follicles Creates a flesh-colored plaque with hypertrichosis No treatment necessary

84. Part 3 What�s the common link?

85. Part 3: Plaques/Nodules

86. Neonatal Dermatology:Part 4 What�s the common link?

88. Nevus Simplex AKA Salmon patch Symmetric localized vascular ectasia most commonly on the glabella, eyelids, upper lip, nuchal area Relatively transient and will usually fade with time (Just say no to grandma!)

90. Acrocyanosis Cyanosis of hands, feet, +/- lips with exposure/chilling Autonomic immaturity?sluggish flow through capillaries?greater oxygen extraction by tissues?higher reduced hemoglobin in capillaries Wrap him up!

92. Harlequin Phenomenon Dramatic, distinct color change involving redness of dependent areas and paleness of upper areas Represents temporarily imbalanced autonomic regulation More common in low birthweight infants

94. Cutis Marmorata Evanescent, lacy, reticulated red/blue diffuse vascular pattern in response to exposure/chilling Represents an accentuated vasomotor response More pronounced CM occurs in Cornelia de Lange, Menke�s, Trisomies 21 and 18 Again, wrap him up!

96. Nevus Flammeus AKA Port-wine stain, stork�s beak mark, capillary hemangioma Permanent vascular malformation of mature dilated dermal capillaries; thicken/darken w/time Macular, sharply circumscribed, various sizes, usually unilateral on head/neck Associated with many syndromes Sturge-Weber (involvement of first branch of trigeminal nerve): glaucoma, leptomeningeal venous angioma, seizures, contralateral hemiparesis, intracranial calcification Also Beckwith-Wiedemann, Klippel-Trenaunay-Weber Complications include traumatic bleeding, poor self-image, hypertrophy of underlying structures Best treatment is pulsed-dye laser

98. Dermal Melanosis AKA Mongolian Spots Homogeneous hyperpigmented round/ovoid macules present at birth Failure of spindle-shaped melanocytes to migrate from deep dermis to epidermis 90% occur in dark-skinned infants over sacrococcygeal, gluteal, and lumbar areas Become less apparent as child ages as the cells eventually migrate to epidermis Differentiate from bruising, abuse, congenital nevi

100. Caf�-au-Lait Macules Uniformly hyperpigmented, sharply demarcated macular lesions with variable size One to three spots in up to 10% infants, considered normal Seen in many disorders Neurofibromatosis I: smooth borders (�Coast of California�) McCune-Albright: irregular borders (�Coast of Maine�) Also Tuberous sclerosis, Russell-Silver, Ataxia-telangectasia, among others

102. Lentigines Small (<3 cm), round dark brown macules anywhere on the body Dense epidermal deposits of melanin Lentiginosis profusa has innumerable small pigmented macules at birth without other abnormalities In children, associated with many syndromes: LEOPARD, Peutz-Jeghers, Gardners In adults, associated with sun damage

104. Ash Leaf Macule Found primarily in tuberous sclerosis, an autosomal dominant disorder with multisystemic involvement Cardiac rhabdomyomas present in 50% at birth, but usually regress without symptoms Other characteristic findings apparent by adolescence including adenoma sebaceum, Shagreen patches, periungual fibromas, epilepsy, renal angiomyolipomas Usually death by third decade

105. Part 4 What�s the common link?

106. Part 4: Isolated changes in color

107. References http://dermatlas.med.jhmi.edu/derm http://www.emedicine.com http://tray.dermatology.uiowa.edu http://health.allrefer.com/health http://www.consultantlive.com Behrman RE, Kliegman RM, Jenson HB: Nelson Textbook of Pediatrics. 17th edition. Philadelphia, Elsevier Science, 2004.