Transient Skin Diseases 1

Disease #1 2

Milia Found on the skin in 40% of infants and on the palate in 60% of infants Known as Epstein’s Pearls when found in the oral cavity Multiple, white, 1 to 2-mm papules seen on the forehead, cheeks and nose Inclusion cysts which contain trapped keratinized stratum corneum Rupture and exfoliate their contents within a few weeks of birth- no treatment required

Sebaceous Gland Hyperplasia 1mm yellow macules or yellow papules Seen at the opening of each pilosebaceous follicle over the nose and cheeks Occur in ~50% of infants Recede by 4 to 6 months of age Pathogenesis: maternal adrogenic stimulation causes increased sebaceous gland volume, cell size, and total number os sebaceous cells

Miliaria Cutaneous changes associated with sweat retention and extravasation of sweet occuring at different levels of the skin Usually due to overheating Miliaria rubra (prickly heat) blocked sweat into the epidermis producing red papulovesicles Miliaria crystallina blocked sweat escapes just beneth the skin surface producing noninflammatory vesicles which look like “clear droplets” 6

Milia Crystallina 7

Disease #2 8

Mottling Lacelike pattern of dusky erythema Appears on the extremities and trunk after exposure to a temperature decrease and disappears on rewarming Pathogenesis: immaturity of the autonomic control of the skin vascular plexus Constriction of the deeper plexus and opening of the superficial plexus Mottling that persists after 6 months may be a sign of hypothyroidism or the vascular malformation cutis marmorata telangiectatia congenita (associated with musculoskeletal or vascular abnormalities)

Disease #3 11

12

Harlequin Color Change Develops when a low-birth-weight infant is placed on one side An erythematous flush with a sharp demarcation at the midline on the dependent side Upper half of the body becomes pale Color change may persist up to 20 minutes after placing the infant supine Pathogenesis: Thought to be due to immaturity of the autonomic vasomotor control 13

Disease #4 14

15

Subcutaneous Fat Necrosis Firm, sharply circumscribed, reddish or purple nodules on cheeks, buttocks, arms, and thighs Usually appear within the 1st weeks of life Spontaneously resolve over several weeks to months Atrophy can occur during healing leading to a depression/ dimpled area Hypercalcemia may occur with or without weight loss, irritability, vomiting, and failure to thrive Differential Diagnosis: Cellulitis or septicemia Pathogenesis: Cold injury 16

Disease #5 17

18

Sucking Blisters Solitary, intact oval blisters or erosions on noninflamed skin Occur on forearms, wrists, fingers, or upper lip Usually resolve within a few days Differenital Diagnosis: Herpesvirus, Bullous impetigo Pathogenesis: Vigorous sucking in utero 19

Disease #6 20

21

Erythema Toxicum Blotchy, erythematous macules 2 to 3 cm in diameter with a tiny 1- to 4- mm central vesicle or pustule Seen on chest, back, face, and proximal extremities Spares the palms and soles Usually begin 24 to 48 hours of age Clear in 4 to 5 days New lesions may appear up to day 10 of life Occur ~50% of term infants Smear of lesions reveal numerous eosinophils 22

Disease #7 23

24

Transient Neonatal Pustular Melanosis Vesicles, pustules, or ruptured vesicles or pustules with a collarette of surrounding scale present at birth Pigmented macules develop at site of resolving lesions Pustules usually disappear by 5 days of age Macules resolve over 3 weeks to 3 months Smear of lesions reveal numerous neutrophils and occasional eosinophil Lesions more common on African American infants Diff Dx: Herpes simplex or bacterial folliculitis Pathogenesis: Unknown c 25

Macules of Resolving Transient Neonatal Pustular Melanosis 26

Pustules in the Newborn 27

Disease #8 28

29

Acne Neonatorum Clinical Findings Appears as multiple, discrete papules at 2 - 4 weeks of life Usually seen on face, chest, back, and groin Papules evolve in pustules after a few weeks Comedones lacking May persist up to 8 months of age Differenital Dx: Erythema toxicum, transient neonatal pustular melanosis, Herpes Simplex, Candidiasis Pathogenesis: Controversial May be a hyposensitivity reaction to Malassezia furfur Treatment: Usually resolves spontaneously 30

Infantile Acne Clinical Findings: Develops ~2-3 months of life Papules, pustules, and open and closed comedones More commonly seen in boys Usually resolved over 6 - 12 months Pathogenesis: Androgenic stimulation of the sebaceous glands Treatment: Usually self resolves Persistant cases may require treatment with topical benzoyl peroxide or antibiotics 31

Disease #9 32

33

Congenital and Neonatal Candidiasis Candida albicans can be acquired prior to birth or during the birth process Infants with congenital disease present at birth with scaling lesions, erythematous papules, and pustules Infants with neonatal disease develop lesions several days or weeks after birth May have diffuse scaling dermatitis or present with typical satellite lesions in intertriginous area Diagnosis made my skin scrapping/ KOH prep Demonstrate pseudohyphae and/or budding yeast Skin culture will demonstate C. albicans 34

Congenital and Neonatal Candisiasis Differenital Dx: Erythema toxicum, miliaria, transient neonatal pustular melanosis, herpes simplex Low birth weight infants can develop systemic candidiasis Pathogenesis: C. albicans can penetrate through the amnion and chorion to cause congenital infection Neonatal infection acquired during the birth process through the vagina colonized with candida Treatment: Topical Antifungal for cutaneous lesions and IV antifugals for systemic disease 35

Disease #10 36

37

Acropustulosis of Infancy Presents as pustules or vesicles on the palms and soles May appear at birth or occur up to age 3 Recurrent crops of erythematous papules that becomes pruritic pustules and resolve with a scale Differenital Dx: Scabies Pathogenesis: Unknown 38

Disease #11 39

40

Herpes Simplex Infection Clinical Features Grouped vesicles on an erythematous base Mean age of onset of lesions 6 days May be present at birth Differential DX: varicella, bullous impetigo Pathogenesis: Herpes Simplex Virus 60-80% Type 2 Infants born to mothers with a primary infection at highest risk Work-Up: Viral culture of skin lesions, urine, nasopharynx, eyes, and CSF Treatment: IV Acyclovir 41

Disease #12 42

43

Varicella Clinical Features: May mimic herpes simplex. Lesions appear in crops of macules and papules that evolve into vesicles and then crust Age of onset in the first 10 days of life Differential DX: herpes simplex, bullous impetigo Pathogenesis: Maternal infection with VZV Treatment: Varicella Immune Globulin if maternal infection is presents from 5 days prior to delivery to 2 days post- partum. nfected infants are treated ith acycolvir. May consider VZIG for premature and term infants exposed postnatally 44

Disease #13 45

46

Omphalitis Clinical Features: Differential DX: Pathogenesis: Redness and induration of the umbilical region Differential DX: Irritant dermatitis secondary to treatment of the umbilicus with bacteriostatic agents Pathogenesis: Bacterial infection through the cut surface of the umbilical cord Predominantly caused by S. aureus If untreated, may lead to sepsis Treatment: IV antistaphylococcal antibiotics 47

Disease #14 48

49

Aplasia Cutis Congenita Clinical Features: Congenital absence of skin Oval, sharply marginated, 1 -2 cm depressed areas Usually occurs in a small, localized area primarily on the posterior scalp Differential DX: Scalp ulcer Pathogenesis: Developmental failure of skin fusion. Dermis, epidermis, and fat may be missing ~25% have skull abnormalities. Consider trisomy 13, if multiple lesions. Treatment: Small lesions corrected with surgical excision. Large lesions may require hair transplantation 50

Vascular Abnormalities 51

Disease #15 52

53

Hemangiomas Clinical Features: Occur in ~2.5% of neonates May resemble a port-wine stain, bruise, or hypopigmented macule Lesions may only be evident by a circumscribed area of blanched skin with a few telangiectases Areas being to becomes raised by 2-4 weeks of age Lesions grows out of proportion to the infant for the 1st 8-12 months of life Usually begin to involute ~15 months Signified by pale gray areas within the nodule Female to Male ratio 3:1 50% will disappear by 5 years and 90% by 9 years 54

Hemangioma Complications Ulceration due to secondary S. aureus superinfection Depends on locations Problematic Locations Periorbital lesions: Astigmatism, amblyopia, refractive error or blindness Beard lesions (Mandible, chin, submental): Subglottic hemangioma- leading to stridor, cough, respiratory difficulty Ear: Risk of obstruction leading to conductive hearing loss Nose/Lips: Greater tendency to ulcerate/ cosmetic deformity Midline lumbosacral region: Increased risk of spinal dysraphism Need an MRI to r/o spinal abnormalitites Multiple cutaneous (>5) hemangiomas: Associated with visceral lesions, especially of the liver 55

PHACE SYNDROME P: Posterior fossa abnormalitites (Dandy Walker Syndrome) H: Hemangiomas (usually large, cervicofacial lesion involving distibution of cranial nerve VI) A: Arterial anomalies (usually intracerebral arterial anomalies) C: Cardiac Defects (especially coartation of the aorta) E: Eye abnormalitites (micro-opthalmia) 56

Hemangiomas Treatment: Usually none Problematic locations may require systemic cortiocosteroids 57

Disease #16 58

59

Dermal Melanosis Clinical Features: Usually present at birth Flat, deep brown to slate gray, or even blue-black Most common sites are lumbosacral region, back, flanks, and shoulders Seen in >90% of African American and Native American infants Occurs <10% of caucasian infants Disappear or fade over years Differential DX: Non-accidental trauma Pathogenesis: Treatment: None required 60

Disease #17 61

62

Supernumerary Nipples Clinical Features: Differential DX: Pathogenesis: Treatment: 63

Disease #18 64

65

Cutis Marmorata Telangietatica Congenita Clinical Features: Differential DX: Pathogenesis: Treatment: 66

Disease #19 67

68

Epidermal Nevi Clinical Features: Differential DX: Pathogenesis: Treatment: 69

Disease #20 70

71

Lymphangioma Clinical Features: Differential DX: Pathogenesis: Treatment: 72

Disease #21 73

74

Sebaceous Nevi Clinical Features: Differential DX: Pathogenesis: Treatment: 75

Disease #22 76

77

Nevus Comedonicus Clinical Features: Differential DX: Pathogenesis: Treatment: 78

Disease #23 79

80

Aplasia Cutis Congenita Clinical Features: Differential DX: Pathogenesis: Treatment: 81

References