Neonatal Dermatology | Figure 2.1
A: Neonatal pustular melanosis*
B: Neonatal cephalic pustulosis
(Reprint from Boekhout T,
Gueho-Kellerman E, Mayser P,
Velegraki A. Malassezia and the
Skin. New York, NY: Springer;
2010)
C: Subcutaneous fat necrosis*
* Reprint from Laxer RM,
ed. The Hospital for Sick
Children: Atlas of Pediatrics.
Philadelphia, PA: Current
Medicine; 2005 |
- Onset at birth; common in darkly pigmented infants
- Presents with small pustules or residual hyperpigmented macules with collarette of scale
- Smear of sterile pustule shows numerous neutrophils
- Histology: subcorneal pustules with neutrophils
Erythema Toxicum Neonatorum- Onset typically 24 – 48 h after birth; occurs in half of all full-term infants
- Presents with blotchy erythematous macules, papules, pustules, and wheals
- Smear of sterile vesicle/pustule shows eosinophils
- Histology: subcorneal pustules with eosinophils, associated with pilosebaceous unit
Neonatal Cephalic Pustulosis (Neonatal Acne) (Figure 2.1B) - Onset typically within first 30 days; Malassezia spp. implicated in pathogenesis
- Presents with erythematous follicular comedones, papules, and pustules on face
- Histology: follicular pustules with neutrophils
Sclerema Neonatorum- Onset usually within first week of life; form of panniculitis in severely ill, premature infants; often fatal
- Presents with diffuse woody hardening of skin; spares genitalia, palms, and soles
- Histology: needle-shaped clefts with necrotic adipocytes with little surrounding inflammation
Subcutaneous Fat Necrosis of the Newborn (Figure 2.1C) - Onset within first weeks of life; localized form of sclerema neonatorum in healthy infants
- Presents with indurated subcutaneous nodules favoring cheeks, shoulders, back, buttocks, and thighs
- Associated with hypothermia, perinatal hypoxemia (from preeclampsia, meconium aspiration, etc.), hypoglycemia
- Calcification may occur; ± profound hypercalcemia with resolution, so prudent to monitor calcium levels until 1 month after full resolution of lesions
- Histology: panniculitis with prominent inflammatory infiltrate, needle-shaped clefts and fat necrosis
Pedal Papules of Infancy- Soft, non-painful papules involving heels
Seborrheic Dermatitis (Figure 2.2A) - Onset typically 1 week after birth; lasts several months, mostly resolves by 1 year of age
- Presents with ill-defined erythematous patches with waxy scale over scalp (“cradle cap”), ± axillae and groin; lesions may appear psoriasiform
Miliaria Crystallina (MC) or Miliaria Rubra (MR)- Onset within first few weeks of life; due to obstructed sweat glands and associated with ↑ temperature (i.e., occlusion)
- Presents with clear vesicles favoring head, neck, and upper trunk (MC) or erythematous papules/vesicles grouped in intertriginous areas or occluded areas (MR)
| Figure 2.2
A: Seborrheic dermatitis
B: ACC, cicatricial (Courtesy of
Dr. Michelle B. Bain)
C: ACC, bullous (Courtesy of
Dr. Michelle B. Bain) |
- Onset before birth; localized defect in epidermis, dermis and/or fat; variable appearance, typically along midline
- Presents with erosion, ulceration, scar, or membranous defect (ovoid lesion covered by an epithelial membrane)
- Hair collar sign: ring of dark long hair encircling lesion; ± marker of underlying neural tube defect
- Typically isolated abnormality, but may be associated with developmental anomalies or following disorders:
| | | | | | | | Bart Syndrome | | ACC of lower extremities + epidermolysis bullosa (dominant dystrophic) | | | Adams–Oliver Syndrome | | ACC on scalp (with skull ossification defect) + extensive CMTC + limb defects (reductions, syndactyly) + cardiac abnormalities | | | Seitles Syndrome | | Bilateral temporal ACC + abnormal eyelashes, “leonine” facies, upward-slanting eyebrows |
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Cutis Marmorata Telangiectatica Congenita (CMTC)- Onset at birth; typically improves with age
- Presents with blanching reticulated vascular pattern on trunk/extremities with segmental distribution
- Associated anomalies in ½ of patients (varicosities, nevus flammeus, macrocephaly, ulceration, hypoplasia, and/or hypertrophy of soft tissue and bone)
Sucking Blister- Onset at birth or soon after; due to sucking
- Presents with solitary blister (hand, wrist, or lip)
Congenital Infections of the Newborn (see Table 2-1)
| | | | | | | Table 2-1 Congenital Infections of the Newborn | | | Infection | | Clinical Findings | | Extracutaneous Findings | | Important Points | | | Cytomegalovirus
(CMV) | | Petechiae, purpura, vesicles, and “blueberry muffin” lesions
(Blueberry muffin lesions: red-blue papules/nodules due to dermal erythropoiesis) | | Intrauterine growth retardation, chorioretinitis, intracranial calcification | | ⇒ Leading infectious cause of deafness and mental retardation
⇒ Typical findings on histology: enlarged endothelial cells with intranuclear inclusions | | | Herpes Simplex
Virus
(HSV) | | Localized or disseminated skin lesions (vesicles, erosions, scarring) | | Encephalitis (predilection for temporal lobes), multi-organ failure, ocular infection | | ⇒ Majority HSV2, 85% acquired perinatally
⇒50–75% mortality if left untreated | | | Rubella | | “Blueberry muffin” lesions | | Cataracts, deafness, congenital heart disease, CNS findings (microcephaly, hydrocephaly), hepatosplenomegaly (HSM) | | ⇒ 50% chance of deafness
⇒ Severe birth defects if within first 16 weeks of pregnancy
⇒ Non-immune pregnant woman transfer the virus to the fetus | | | Toxoplasmosis | | “Blueberry muffin” lesions favoring the trunk | | Ocular abnormalities (chorioretinitis, blindness), CNS abnormalities (deafness, mental retardation, seizures), thrombocytopenia, intracranial calcification | | | | | Varicella | | Cicatricial skin lesions | | Ocular abnormalities (chorioretinitis, cataracts), cortical atrophy, psychomotor retardation, hypoplastic limbs | | ⇒ Greatest risk in first 20 weeks
⇒ 2% risk of embryopathy in women with infection within first two trimesters | | | Syphilis, Early
Congenital | | Syphilitic pemphigus, rhagades (radial furrows/fissures in perioral area, turn into parrot lines), papulosquamous macules/papules (like secondary syphilis) | | Snuffles (rhinitis, secondary to ulcerated mucosa), enlarged lymph nodes and spleen, neurosyphilis
(Be able to differentiate early and late congenital syphilis findings) | | ⇒ Early congenital syphilis occurs from birth to 2 years of age
⇒ Only congenital syphilis may show bullous lesions
⇒ Papulosquamous lesions common in the diaper area | | | Syphilis, Late
Congenital | | Hutchinson’s teeth, Higoumenakis sign, mulberry molars, saddle nose, saber shins, parrot lines and furrows | | Interstitial keratitis, gummas along long bones/skull, tabes dorsalis, generalized paresis | | ⇒ Includes permanent sequelae of early congenital signs
⇒ Higoumenakis sign: congenital thickening of the medial aspect of the clavicle | | | | | | | | | |
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Differential Diagnosis of ‘Diaper Dermatitis’ (see Table 2-2)
| | | | | | | Table 2-2 Differential Diagnosis for Diaper Dermatitis | | | Entity | | Clinical Findings | | | Candidal Dermatitis | | Bright red patches with pustules and satellite papules, ± intertriginous involvement (including scrotum), ± thrush | | | Irritant Dermatitis | | Poorly demarcated erythematous plaques, spares inguinal folds | | | Seborrheic Dermatitis | | Typical salmon-covered scaly patches and plaques involving the scalp, groin, and other intertriginous areas | | | Psoriasis | | Sharply demarcated bright pink to red plaques involving inguinal creases, minimal scale; most common psoriatic presentation in infants | | | Allergic Contact Dermatitis | | Rare in infants, ± related to topical preparations or foods | | | Atopic Dermatitis | | Increased incidence of diaper dermatitis in atopic patients | | | Miliaria | | Clear vesicles or erythematous papules/pustules due to blocked
eccrine ducts from heat or humidity in diaper area | | | Granuloma Gluteale Infantum | | Red to violaceous granulomatous nodules over the vulva, perianal area, buttocks, ± scrotum; due to irritation, occlusion, candidal infection | | | Perianal Pseudoverrucous Nodules | | Erythematous nodules and papules in children with fecal incontinence | | | Acrodermatitis Enteropathica | | Erythematous crusted patches/plaques with flaccid bullae in perineal, periorificial, and distal extremities; due to ↓ zinc level (also ↓ alkaline phosphatase as zinc-dependent); may occur in following settings: | | | | | - Premature infants (poor absorption and ↑ requirement of zinc) when weaned off breast milk (which has adequate zinc level)
- Inherited form (AR) manifests when weaned off breast milk
- Healthy infants if low zinc level in maternal milk
- Acquired form if malabsorption or inadequate nutrition
| | | Cystic Fibrosis | | Resembles acrodermatitis enteropathica, also due to zinc deficiency ± pedal edema, failure to thrive, infections and malabsorption | | | Multiple Carboxylase Deficiency | | Both resemble acrodermatitis enteropathica (periorificial dermatitis); treatment for both forms (listed below) is biotin | | | Biotin Deficiency | | - Neonatal form: AR, holocarboxylase synthetase deficiency, ± erythroderma with alopecia, fatal if not treated
- Juvenile form: biotinidase deficiency, ± seizures, alopecia, hearing loss, developmental delay
| | | Langerhans Cell Histiocytosis | | Yellow-brown crusted papules with purpura in seborrheic distribution; ± systemic involvement; Langerhans cells (CD1a +, S100+) | | | Kawasaki Disease | | Tender erythema in perineal area which later desquamates | | | Perianal Strep | | Bright red, well-demarcated perianal erythema and involving creases | | | Bullous Impetigo | | Honey-colored crusts and flaccid bullae | | | Scabies | | Erythematous nodules involving diaper area, ± genitalia | | | Congenital Syphilis | | Reddish-brown papulosquamous eruption, may be erosive or bullous | | | | | |
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