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
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
Transient Neonatal Pustular Melanosis (Figure 2.1A)
  • 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)
Figure 2.2
A: Seborrheic dermatitis
B: ACC, cicatricial (Courtesy of
Dr. Michelle B. Bain)
C: ACC, bullous (Courtesy of
Dr. Michelle B. Bain)
Aplasia Cutis Congenita (ACC) (Figure 2.2B, C)
  • 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
 
   

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
        
 
   

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:
 
  1. Premature infants (poor absorption and ↑ requirement of zinc) when weaned off breast milk (which has adequate zinc level)
  2. Inherited form (AR) manifests when weaned off breast milk
  3. Healthy infants if low zinc level in maternal milk
  4. 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
  1. Neonatal form: AR, holocarboxylase synthetase deficiency, ± erythroderma with alopecia, fatal if not treated
  2. 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