Vesiculopustular and Erosive Disorders in Newborns and Infants

Abstract

A wide variety of conditions can cause vesicles, pustules, bullae, erosions, and ulcerations during the newborn period and infancy. Accurate and prompt diagnosis is important because some of the underlying disorders represent potentially life-threatening infections; conversely, many are benign and self-limited. Therefore, it is essential to develop a systematic approach to the evaluation and treatment of newborns and infants with these types of skin lesions. This chapter outlines the differential diagnosis and initial management of the most common and important vesiculopustular and erosive disorders in newborns and infants.

Keywords

erythema toxicum neonatorum, transient neonatal pustular melanosis, neonatal cephalic pustulosis, eosinophilic pustular folliculitis of infancy, acropustulosis of infancy, vesicles, pustules, bullae, erosions, ulcers

A wide variety of conditions can cause vesicles, pustules, bullae, erosions, and ulcerations during the newborn period and infancy. Accurate and prompt diagnosis is important because some of the underlying disorders represent potentially life-threatening infections; conversely, many are benign and self-limited. Therefore, it is essential to develop a systematic approach to the evaluation and treatment of newborns and infants with these types of skin lesions. An algorithm outlining initial and subsequent investigations is presented in Fig. 34.1 . This chapter highlights many causes of vesiculopustular and erosive eruptions during the first year of life, and more exhaustive lists are provided in Tables 34.1 and 34.2 . The majority of infectious etiologies are also covered in Chapter 74 , Chapter 75 , Chapter 76 , Chapter 77 , Chapter 78 , Chapter 79 , Chapter 80 , Chapter 81 , Chapter 82 .

Table 34.1

Differential diagnosis of vesiculopustular diseases.

Additional etiologies include irritant contact dermatitis (see Table 34.2 ), dermatophyte infection, Sweet syndrome, and intrauterine coxsackievirus or Chikungunya virus infection. The vesicular eruption of lipoid proteinosis tends to develop after 1 year of age (see Ch. 48 ). AD, autosomal dominant; AR, autosomal recessive; CNS, central nervous system; DFA, direct fluorescent antibody; HSV, herpes simplex virus; KOH, potassium hydroxide examination; PCR, polymerase chain reaction.

Adapted from Frieden IJ, Howard R. In: Eichenfield LF, Frieden IJ, Mathes EF, Zaenglein AL (eds). Neonatal and Infant Dermatology. London: Elsevier, 2015:112–115.

DIFFERENTIAL DIAGNOSIS OF VESICULOPUSTULAR DISEASES
Disease	Usual age of onset	Skin: morphology	Skin: distribution	Diagnostic studies (skin)	Comments
Infectious diseases (more common conditions are in bold )
Staphylococcal diseases: pyoderma and bullous impetigo (see Ch. 74 ) ( Fig. 34.3A )	Few days to weeks or older	Pustules, bullae, occasionally vesicles; superficial erosions with collarettes of scale, crusts; furuncles	Favors diaper and periumbilical areas	Gram stain: Gram-positive cocci in clusters; bacterial culture	May occur in outbreaks
Group A streptococcal infection (see Ch. 74 )	Few days to weeks or older	Isolated pustules, honey-colored crusts; moist, foul-smelling, fiery red intertriginous erythema	Any body region; may affect moist umbilical stump or skin folds	Gram stain: Gram-positive cocci in chains; rapid antigen test; bacterial culture	Occasionally cellulitis, meningitis, pneumonia
Group B streptococcal infection	Birth to first few days	Vesicles, bullae, erosions, pustules, honey-colored crusts	Any body region	Gram stain: Gram-positive cocci in chains; bacterial culture	Pneumonia, bacteremia, meningitis
Listeria monocytogenes infection (see Ch. 74 )	Birth to first few hours	Hemorrhagic pustules and petechiae	Generalized, especially trunk and extremities	Gram stain: Gram-positive rods; bacterial culture	Sepsis; respiratory distress; history of maternal fever and premature labor
Haemophilus influenzae infection (see Ch. 74 )	Birth to first few days	Vesicles, crusted papules	Any body region	Gram stain: Gram-negative bacilli; bacterial culture	Bacteremia, meningitis
Pseudomonas aeruginosa infection (see Ch. 74 )	Days to weeks or older	Erythema, pustules, hemorrhagic bullae, necrotic ulcerations	Any region, especially anogenital area	Gram stain: Gram-negative rods; bacterial culture	Prematurity, low birth weight, immunodeficiency
Congenital candidiasis ( Figs 34.3B & 34.7 )	Birth to first few days	Erythema, small papules and pustules, fine scaling; “burn-like” if extremely premature (see Table 34.2 )	Any region; palms/soles often involved; nails may be affected	KOH: budding yeast; fungal culture; placental/umbilical cord lesions may be present	Prematurity, foreign body in cervix/uterus are risk factors; ascending in utero infection
Neonatal candidiasis	1–2 weeks of age	Beefy red patches with scale; satellite pustules and papules	Diaper area, other intertriginous zones, face	KOH: budding yeast, pseudohyphae; fungal culture	Usually otherwise healthy; acquisition during delivery or postnatally; oral thrush
Aspergillus infection (see Ch. 77 )	Few days to weeks	Pustules (often clustered) rapidly evolve to necrotic ulcers	Any region, especially macerated skin in the diaper area and under adhesive tape/occlusive dressings	Skin biopsy: septate hyphae; tissue fungal culture	Extreme prematurity
Neonatal HSV infection (see Ch. 80 ) ( Fig. 34.14 )	Birth to 2 weeks, usually after 5 days	Vesicles, pustules, crusts, erosions	Any region, especially scalp, torso; may involve mucosa	Tzanck preparation, PCR, DFA or immunoperoxidase slide test, viral culture	Signs of sepsis; irritability, lethargy; must exclude herpes meningitis/encephalitis
Intrauterine HSV infection	Birth	Vesicles, pustules, widespread erosions, scars, areas of absent skin	Any body region	Tzanck preparation, PCR, DFA or immunoperoxidase slide test, viral culture	Low birth weight, microcephaly, chorioretinitis
Neonatal varicella (see Ch. 80 )	Birth to 2 weeks	Vesicles on an erythematous base	Generalized distribution	Tzanck preparation, PCR, DFA, viral culture	Maternal primary varicella infection 7 days before to 2 days after delivery
Herpes zoster (see Ch. 80 )	Typically 2 weeks or older	Vesicles on an erythematous base	Dermatomal pattern	Tzanck preparation, PCR, DFA, viral culture	Maternal primary varicella during pregnancy or up to a few days after delivery, or neonatal varicella
Scabies (see Ch. 84 )	Typically 3–4 weeks or older	Papules, nodules, crusted areas, vesicles, pustules, burrows	Any region; especially axillae, groin, palms/soles, and wrists	Mineral oil preparation; dermoscopy	Family members may have pruritus and similar lesions
*Common transient conditions*
Erythema toxicum neonatorum ( Fig. 34.2 )	Typically 24–48 hours but can be birth to 2 weeks	Erythematous macules, papules, pustules > vesicles, wheals	Any region, except almost always spares palms/soles	Clinical; Wright’s stain: eosinophils	Term infants >2500 g
Transient neonatal pustular melanosis ( Fig. 34.4 )	Birth	Pustules without erythema; collarettes of scale; hyperpigmented macules	Any region; most often forehead, neck, lower back, shins; may affect palms/soles	Clinical; Wright’s stain: neutrophils, occasional eosinophils, cellular debris	Term infants; more common in infants of African descent
Miliaria crystallina (see Ch. 39 ) ( Fig. 34.5A )	Birth to early infancy	Fragile vesicles without erythema	Forehead, upper trunk, arms most common	Clinical	Sometimes history of overheating or fever
Miliaria rubra (see Ch. 39 ) ( Fig. 34.5B )	Typically ≥1 week	Erythematous papules with superimposed pustules	Forehead, neck, upper trunk; occluded areas most common	Clinical; Wright’s stain: variable inflammatory cells but not prominent eosinophils	Sometimes history of overheating or fever
Neonatal cephalic pustulosis (neonatal “acne”) ( Fig. 34.6 )	~5 days to 3 weeks	Papules and pustules on erythematous base	Cheeks, forehead, chin, eyelids; less commonly neck, upper chest, scalp	Clinical; Giemsa stain: yeast forms, neutrophils	Otherwise well
*Uncommon and rare non-infectious diseases*
Acropustulosis of infancy ( Fig. 34.10 )	Typically 3–6 months, occasionally birth to weeks	Vesicles and pustules	Hands and feet; occasionally scalp, trunk	Clinical; assess for scabies infestation; skin biopsy: intraepidermal vesicle/pustule with neutrophils and occasionally eosinophils	Severe pruritus; lesions recur in crops; subset with prior scabies
Eosinophilic pustular folliculitis of infancy ( Fig. 34.11 )	Birth to 14 months, mean 6 months	Papules and pustules	Scalp > face > trunk, extremities	Skin biopsy: dense mixed infiltrate with eosinophils, often but not invariably centered on hair follicles	Pruritus; lesions recur in crops; often peripheral eosinophilia; neonatal eosinophilic pustulosis variant favors the face in premature boys
Congenital and neonatal Langerhans cell histiocytosis (see Ch. 91 ) ( Fig. 34.12 )	Birth to weeks	Vesicles, crusts, papules, nodules, petechiae	Any body region, especially flexural sites, palms/soles, scalp	Skin biopsy: S100 ⁺/CD1a ⁺histiocytes with reniform nuclei, focal invasion of epidermis	Occasional mucosal or extracutaneous involvement; pure cutaneous form often resolves spontaneously, but later cutaneous and systemic relapses possible
Incontinentia pigmenti (see Ch. 62 ) ( Fig. 34.13 )	Birth to weeks	Vesicles, hyperkeratotic papules along the lines of Blaschko	Vesicular lesions most common on the extremities	Skin biopsy: eosinophilic spongiosis with necrotic keratinocytes; genetic analysis ( IKBKG / NEMO )	Ocular, CNS, and dental involvement common but often not evident at birth; X-linked dominant, patients usually female
Autosomal dominant hyper-IgE syndrome (see Ch. 60 )	Birth to weeks	Single and grouped vesicles or papulopustules	Face, scalp, upper trunk, axillae, diaper area	Skin biopsy: intraepidermal vesicle with eosinophils, eosinophilic folliculitis; genetic analysis ( STAT3 )	Eosinophilia with variably elevated IgE levels; abscesses, pneumonias, and pneumatoceles often develop after neonatal period
Vesiculopustular eruption of transient myeloproliferative disorder in Down syndrome	Days to weeks	Vesicles and pustules	Face > trunk, extremities; sites of adhesive dressings, minor trauma	Intraepidermal spongiotic vesiculopustules, infiltrate containing immature myeloid cells	Trisomy 21 or mosaicism for trisomy 21; severe leukocytosis with immature myeloid cells; increased risk of myeloid leukemia
Erosive pustular dermatosis of the scalp	Weeks to months	Pustules, erythema with scale-crust, erosions; alopecia and scarring	“Halo scalp ring” pattern, vertex of scalp	Clinical; skin biopsy: alopecia, scarring, mixed dermal infiltrate	Prolonged labor and delivery; necrotic caput succedaneum at birth
Neonatal Behçet disease (see Ch. 26 )	First week	Vesiculopustular, purpuric and necrotic skin lesions; oral and genital ulcers	Lesions favor hands and feet as well as oral and genital mucosae	Clinical	Maternal history of Behçet disease; diarrhea, vasculitis
Pustular psoriasis, including deficiency of interleukin-36 receptor antagonist (DITRA; AR) and CARD14-associated pustular psoriasis (CAMPS; AD) (see Chs 8 and 45 )	Weeks to months or older	Pustules and pustular lakes within areas of erythema	Often generalized with erythroderma; pustules in any body region, especially the palms/soles	Skin biopsy: spongiform pustules and microabscesses within the epidermis, parakeratosis, dilated dermal capillaries; consider genetic analysis ( IL36RN , CARD14 )	Occasional fever; often resistant to therapy
Deficiency of interleukin-1 receptor antagonist (DIRA; see Ch. 45 )	Birth to weeks	Pustules within areas of erythema	Often generalized with erythroderma; oral lesions	Skin biopsy: neutrophilic microabscesses within acanthotic epidermis, parakeratosis, dilated dermal capillaries; genetic analysis ( IL1RN )	Sterile osteolytic or hyperplastic bone lesions, neonatal distress; dramatic response to IL-1 antagonists
Perforating neutrophilic and granulomatous dermatitis associated with immunodeficiency	Birth to weeks	Papules evolve into vesicles, pustules, crusts, and ulcers	Varies: face, extremities, perineum	Skin biopsy: granulomas, neutrophilic infiltrate, transepidermal elimination of degenerated collagen and debris through hair follicles	Primary immunodeficiencies, including APLAID (autoinflammation and phospholipase C γ2-associated antibody deficiency and immune dysregulation)

Table 34.2

Differential diagnosis of bullae, erosions and ulcerations.

AD, autosomal dominant; AR, autosomal recessive; CNS, central nervous system; CSF, cerebrospinal fluid; DEJ, dermal–epidermal junction; DFA, direct fluorescent antibody; DIF, direct immunofluorescence; FDP, fibrin degradation product; GVHD, graft-versus-host disease; PT, prothrombin time; PTT, partial thromboplastin time.

Adapted from Frieden IJ, Howard R. In: Eichenfield LF, Frieden IJ, Mathes EF, Zaenglein AL (eds). Neonatal and Infant Dermatology. London: Elsevier, 2015:112–115.

DIFFERENTIAL DIAGNOSIS OF BULLAE, EROSIONS AND ULCERATIONS
Disease	Usual age	Skin: morphology	Skin: distribution	Diagnostic studies (skin)	Comments
Infectious diseases
Staphylococcal scalded skin syndrome (see Ch. 74 ) ( Fig. 34.17 )	Few days to weeks; rarely congenital	Erythematous patches, fragile bullae, superficial erosions, peeling in sheets	Generalized with periorificial and intertriginous accentuation	Biopsy: epidermal separation at granular cell layer; culture positive only at primary site(s) of infection (toxin-mediated)	Irritability, temperature instability; high risk of secondary sepsis, fluid/electrolyte abnormalities
Group B streptococcal infection	(see Table 34.1 )
Pseudomonas aeruginosa infection	(see Table 34.1 )
Congenital syphilis (see Ch. 82 )	Birth to first few days	Bullae or erosions; erythema and desquamation	Any region, especially perioral, palms/soles	Darkfield examination of serous exudates, DFA; serologic studies (treponemal and non-treponemal)	Snuffles, hepatosplenomegaly, periostitis with pseudoparalysis
“Invasive fungal dermatitis” due to candidiasis > other fungi (see text and below) in very-low-birth-weight premature newborns ( Fig. 34.7 )	Birth to 2 weeks	“Burn-like” erythema with desquamation and erosions; intertriginous maceration	Any region	KOH: budding yeast; fungal culture; placental/umbilical cord lesions may be present	Risk factors: birth weight <1000 g, vaginal delivery, systemic corticosteroid administration, prolonged hyperglycemia; high risk of disseminated systemic infection
Aspergillus infection	(see Table 34.1 )
Zygomycosis, trichosporonosis (see Ch. 77 )	Days to weeks	Generalized peeling and skin breakdown or cellulitis evolving into necrotic ulcer	Any body region	Skin biopsy and tissue fungal culture	Extreme prematurity
Intrauterine HSV infection	(see Table 34.1 )
Congenital (fetal) varicella syndrome (see Ch. 80 )	Birth	Erosions, ulcers, scarring, aplasia cutis congenita	Any region, especially extremities; may have dermatomal pattern	Variably positive Tzanck preparation, PCR, DFA, viral culture	Maternal varicella in the first or early second trimester; limb atrophy, CNS/eye abnormalities
*Conditions with exogenous causes*
Sucking blisters ( Fig. 34.8 )	Birth	Flaccid bulla or linear erosion; most often solitary	Radial forearm, wrist, hand, fingers, occasionally foot	Clinical	Due to sucking on affected areas in utero
Irritant contact dermatitis	First few days to weeks or older	Glazed erythema, vesiculation, punched-out erosions	Diaper area, especially on convex surfaces	Usually clinical	Severe form referred to as Jacquet erosive diaper dermatitis
Perinatal trauma/iatrogenic injury	Birth or neonatal period	Erosions, ulcerations, scars, atrophic lesions	Depends on cause of trauma	Usually clinical	Perinatal history of fetal scalp electrode monitor, prolonged labor and/or vacuum or forceps delivery, history of amniocentesis, etc.
Perinatal gangrene of the buttock	Days	Sudden-onset erythema and cyanosis, then gangrenous ulcers	Buttocks	Clinical	Umbilical artery catheterization in some cases
*Associated with birthmarks and related conditions*
Erosions overlying large congenital melanocytic nevi (see Ch. 112 )	Birth to first few days	Erosions, ulcerations	Superimposed on the congenital melanocytic nevus, most often on the back	Clinical; skin biopsy to exclude melanoma if persistent or other unusual features	Neurocutaneous melanosis present in subset of patients
Infantile hemangioma (see Ch. 103 )	Birth to first few weeks or months	Ulceration, often with a bright red border	Any site, but diaper area and lip most common	Clinical	Cause often not clinically apparent until hemangioma begins to proliferate
Aplasia cutis congenita (see Ch. 64 ) ( Fig. 34.9 )	Birth	“Bullous” form: round/oval, sharply demarcated with overlying membrane; other types with raw, full-thickness ulcer; may occasionally present as scar	Scalp (especially vertex) or face most common; other sites depending on etiology	Usually clinical; imaging studies to evaluate underlying bone, CNS	Possible associations: CNS defects, sagittal sinus thrombosis, trisomy 13, limb-reduction abnormalities
Linear porokeratosis, porokeratotic eccrine ostial and dermal duct nevus (PEODDN) (see Chs 62 , 109 , & 111)	Birth or weeks to years later	Occasionally eroded at birth; usually erythema and keratotic border or (in PEODDN) spines	Often extremities, but any site possible	Skin biopsy: cornoid lamella (may not be evident in newborn period)	Risk of squamous cell carcinoma; mosaic GJB2 mutations in PEODDN
*Uncommon and rare non-infectious diseases*
Mastocytosis (see Ch. 118 ; Fig. 34.18 )	Birth to weeks or months	Localized/discrete lesions : papules, plaques or nodules with intermittent superimposed wheals, bullae, erosions; often hyperpigmented Diffuse form : blistering and erosions superimposed on infiltrated skin with “peau d’orange” appearance	Any body region	Positive Darier sign; skin biopsy: increased mast cells in dermis	Variably present: flushing, irritability, diarrhea, abdominal pain
Epidermolysis bullosa (see Ch. 32 )	Birth to first few days or older	Mechanically induced blisters and erosions; depending on type: mucosal erosions, aplasia cutis congenita of anterior legs, scarring, milia, nail dystrophy	Widespread or limited, depending on type; most often extremities, especially hands/feet	Biopsy of induced blister for immunofluorescence antigen mapping ± electron microscopy; genetic analysis	Difficulty feeding, failure to thrive; occasionally corneal, respiratory tract or gastrointestinal (e.g. pyloric atresia) involvement; anemia
Kindler syndrome (see Ch. 32 )	Birth to weeks or older	Trauma-induced blisters and erosions; progressive poikiloderma	Bullae most common in acral sites; poikiloderma is widespread with skip areas	Skin biopsy: immunostaining with anti-kindlin-1 antibody; genetic analysis ( FERMT1 )	Photosensitivity; gingivitis, colitis, mucosal stenoses (e.g. esophageal, urethral); acquired syndactyly (toes, proximal fingers); AR inheritance
Epidermolytic ichthyosis (see Ch. 57 ; Fig. 34.16 )	Birth	Erythroderma with blisters and erosions	Generalized	Skin biopsy: epidermolytic hyperkeratosis; genetic analysis ( KRT1 , KRT10 )	Risk of sepsis, fluid and electrolyte imbalances; AD inheritance
Maternal autoimmune bullous disease	Birth	Depends on type of maternal disease: tense or flaccid bullae, erosions	Variable; often widespread	Skin biopsy with DIF usually diagnostic	Maternal history of blistering disease, but occasionally inactive during pregnancy
Bullous pemphigoid (see Ch. 30 )	Usually 2 months of age or older	Tense bullae	Favor hands and feet but may be generalized	Skin biopsy: subepidermal bullae with eosinophils; DIF: linear pattern IgG at DEJ
Linear IgA bullous dermatosis (see Ch. 31 )	Rarely at birth, usually later infancy or childhood	Tense blisters often form rosette or sausage shapes	Widespread but often concentrated in the girdle area; usually spares mucosa	Skin biopsy: subepidermal bullae with neutrophils; DIF: linear pattern IgA at DEJ
Neonatal (congenital) lupus (see Ch. 41 )	Birth (~20%) to weeks or months	Congenital erosions/ulcers, crusting, rarely bullae as well as classic annular erythematous plaques; atrophy, scarring, background of livedo reticularis	Face favored, especially the periorbital area, but can be more widespread	Skin biopsy: epidermal atrophy, vacuolar interface dermatitis, variable mucin	Mother and infant with antibodies to SSA/Ro, SSB/La, and/or U1RNP; variable heart block, cardiomyopathy, hepatobiliary disease, cytopenias
Toxic epidermal necrolysis (TEN; see Ch. 20 ) or TEN-like GVHD (see Chs 52 , 60 )	Usually 6 weeks of age or older, except for intrauterine GVHD	Tender erythema, bullae, epidermal sloughing/erosions	Often widespread, evolving rapidly over hours to days; mucosal involvement	Skin biopsy: subepidermal blister with epidermal necrosis (usually full thickness)	Typically in setting of Gram-negative sepsis or due to maternofetal or transfusion-associated GVHD in infants with severe combined immunodeficiency
Intrauterine epidermal necrosis	Birth	Widespread erosions and ulcerations without vesicles or pustules	Generalized, spares mucous membranes	Skin biopsy: epidermal necrosis and calcification of pilosebaceous units	Prematurity; brain infarcts, cardiomegaly, renal tubular necrosis; rapid mortality
Congenital erosive and vesicular dermatosis ( Fig. 34.15 )	Birth	Erosions, vesicles, crusts, “scalded skin”	Generalized with relative sparing of face, palms/soles	Clinical diagnosis, often retrospective; skin biopsy (nonspecific): epidermal necrosis, neutrophils or mixed infiltrate; must exclude other etiologies (e.g. HSV)	Prematurity; heals with supple reticulated scarring; CNS/developmental abnormalities
Pyoderma gangrenosum (see Ch. 26 )	Rare reports of congenital or neonatal onset	Sharply demarcated ulcerations with undermined borders	Any site, especially groin and buttocks	Clinical; exclusion of other etiologies; skin biopsy: infiltration of neutrophils without primary vasculitis or infection	Associations include inflammatory bowel disease, chronic recurrent multifocal osteomyelitis; consider immunodeficiency and autoinflammatory syndromes
Acrodermatitis enteropathica * (see Ch. 51 )	Weeks to months	Sharply demarcated, eroded erythematous plaques with scale-crust; occasionally intact vesicles/bullae	Periorificial (i.e. around mouth, nose, eyes, anus, genitalia), neck folds, hands/feet	Low serum zinc and alkaline phosphatase levels	Irritability, diarrhea, failure to thrive; onset classically after weaning if breastfed; acquired zinc deficiency: low maternal breast milk zinc or prematurity; genetic form with AR inheritance ( SLC39A4 )
Methylmalonic acidemia, other organic acidemias/aminoacidopathies * (see Fig. 51.13 )	Days to weeks	Erythema with or without erosions	Periorificial or more widespread	Urine organic acid analysis; plasma isoleucine level (if restricted diet)	Lethargy, hypotonia, neutropenia, metabolic acidosis; in some cases, skin findings result from therapeutic restrictions of dietary amino acids
Restrictive dermopathy	Birth	Rigid tense skin with linear erosions, tears	Generalized; skin tears most common in flexural creases	Clinical; distinguish from Neu–Laxova syndrome (intrauterine growth retardation, microcephaly, abnormal brain development, edema, parchment-like or ichthyosiform skin); genetic analysis ( LMNA, ZMPSTE24 )	Joint contractures, micrognathia, fixed facial expression, restrictive pulmonary disease; early mortality
Focal dermal hypoplasia (see Ch. 62 )	Birth	Occasional blisters, but more often hypoplasia or aplasia of skin; linear and whorled pattern; also fat “herniation”, telangiectasias	Any body region	Clinical; skin biopsy: dermal hypoplasia with fat abutting epidermis; genetic analysis ( PORCN )	Skeletal, eye and CNS abnormalities to varying degrees
Absent dermal ridges and congenital milia (Basan syndrome; see Ch. 64 )	Birth	Bullae; absent dermal ridge patterns, multiple milia	Fingers, plantar surface of feet	Clinical; genetic analysis ( SMARCAD1 )	AD inheritance
Porphyrias (see Ch. 49 )	Days to months	Photosensitive erythema, blistering, erosions; scarring	Sun-exposed areas or more generalized if phototherapy for hyperbilirubinemia	Transient form: elevated plasma porphyrins; heritable forms: elevated urine, fecal and/or plasma porphyrins ± pink/fluorescent urine	Transient form usually related to hemolytic disease; rare heritable forms present with blistering in infancy, e.g. congenital erythropoietic porphyria
Neonatal purpura fulminans	Days	Initially purpura or cellulitis-like areas evolving to necrotic bullae or ulcers	Buttocks, extremities, trunk and scalp	Prolonged PT/PTT, low fibrinogen, elevated FDPs, low protein C or S levels	Related to sepsis or inherited protein C or S deficiency
Ankyloblepharon–ectodermal dysplasia–clefting (AEC) syndrome (see Ch. 63 )	Birth	Erythroderma, peeling skin, superficial erosions	Generalized, with prominent scalp involvement	Clinical; genetic analysis ( TP63 )	Associated findings include ankyloblepharon, ectodermal dysplasia, and cleft lip/palate