Neonatal dermatoses: Some are cause for more concern than others

Wailea, Hawaii - The level of concern for neonatal dermatoses ranges depending on the type of condition, according to Julie V. Schaffer, M.D., director of pediatric dermatology, director of resident education, assistant professor of pediatrics and dermatology, NYU Langone Medical Center, New York.

Dr. Schaffer spoke on this topic at the 2010 MauiDerm: Advances in Cosmetic and Medical Dermatology in January.

“Common neonatal dermatoses include a variety of conditions, some worthy of more concern while others resolve,” she says.

Here are some conditions and their level of worry:

• Erythema toxicum neonatorum affects about half of full-term neonates and has a typical onset of 24 to 48 hours after delivery (ranges from birth to 2 weeks). The condition can occur on any site, but tends to spare palms and soles.

“There are no absolutely no worries with this,” she says, noting that it will resolve on its own in five to seven days.

• Congenital candidiasis presents with papulopustules and desquamation usually at birth, but there could be a possible delay up to a few days. It is also described as "burn-like" in premies and can be widespread, appearing on the palms and soles often and in the diaper area. The oral mucosa is typically spared.

“Risk factors include maternal vaginal candidiasis, maternal foreign body and prematurity,” she says. “If the baby is full-term and otherwise well, there are no worries.”

Treatment is topical imidazole therapy. However, if the baby is full-term and not otherwise well, for example with respiratory distress, there is cause for some concern and treatment is a systemic antifungal, she notes. For a premie, especially <1,500 g, the concern is high and there is a high risk of disseminated candidiasis, which usually warrants an IV antifungal.

• Transient neonatal pustular melanosis affects about 5 percent of black and about 0.5 percent of white neonates. It is usually evident at birth.

“Sometimes the only symptom is brown, ‘freckle-like’ macules. Although any site can be affected, there is a predilection for the forehead, neck and diaper area. This is a cause for no worries,” Dr. Shaffer says.

• Bullous impetigo is caused by the local production of exfoliative toxin (cleaves Dsg1) by Staphylococcus aureus. This may look alarming, but is really cause for little worry. If very localized it can be treated with topical or systemic antibiotics, Dr. Shaffer notes.

• Staphylococcal scalded skin syndrome (SSSS) is characterized by periorificial crusting, radial fissures, edema, sparing of vermilion lip and mucosa. It presents days to weeks after birth and is usually caused by the systemic elaboration of S. aureus exfoliative toxin.

“This is cause for some worry because there is a risk of sepsis, fluid/electrolyte abnormality,” Dr. Shaffer says. Treatment includes parenteral antibiotic therapy.

• Neonatal cephalic pustulosis, or neonatal acne, has an onset within first few weeks. It usually presents with papulopustular eruption on the face, especially the cheeks, forehead and may extend to scalp, neck and upper trunk (sometimes overlapping with seborrheic dermatitis).

“This condition resolves spontaneously over a few weeks. Topical imidazole can be used but is not necessary,” Dr. Shaffer says.

• Transient myeloproliferative disorder is associated with Trisomy 21 ("full" or mosaic) and affects 10 percent of babies with the condition. Onset is in first two weeks of life with vesiculopustular eruption, usually on the face and on sites of trauma (venipuncture, contact with nasal canula or adhesive), and pseudocellulitis.

“There is cause for some concern because there is an increased risk of megakaryocytic AML,” she notes.

• Congenital Langerhans cell histiocytosis presents at birth.

“Lesions may continue to develop over days-weeks and crusted papules, eroded nodules and vesiculopustules are most common,” Dr. Shaffer says, noting that there is often a variable extent of involvement from solitary to widespread skin lesions. There may also be systemic involvement, including liver, bone marrow and bones.

“Evaluation includes LFTs, CBC/differential, skeletal survey,” she says. “If the disease is limited to the skin, there is little cause for concern. Lesions usually resolve spontaneously over weeks to months. However, if there is systemic disease it is cause for significant concern and the prognosis is less favorable. This requires systemic chemotherapy."

• Neonatal lupus erythematosus (NLE) is caused by maternal anti-Ro antibodies and it is not of significant concern with regard to the first child.

“The risk of NLE is about 1 to 2 percent," Dr. Shaffer says. However, there is more concern if a previous child was affected, and risk increases to about 25 percent.

“New extracutaneous considerations include CNS (hydrocephalus (~10 percent) and macrocephaly (~15 percent at age 8-24 months); and rhizomelic chondrodysplasia punctata phenotype."

• "Stellate" aplasia cutis congenita (ACC) is related to vascular abnormalities or intrauterine ischemic events, including Adams-Oliver syndrome and fetus papyraceus.

“There is more worry for scalp lesions because there is a higher likelihood of underlying skull/vascular defects and also risk of life-threatening sagittal sinus hemorrhage/thrombosis, and meningitis,” Dr. Shaffer says, noting that early surgical repair is often required.

• Intrauterine and neonatal herpes simplex virus (HSV) presents at birth for intrauterine HSV, and at birth to 2 weeks but usually after five days for neonatal HSV. Presentation includes vesicles, pustules and erosions. If intrauterine, there are scars and areas of absent skin. Any site can be affected, including mucosa. Neonatal HSV favors the scalp and trunk. Concerns exist for CNS, eye and disseminated disease are possible, she says.

• Babies with congenital erosive and vesicular dermatosis (CEVD) are usually born prematurely and have widespread erosions, vesicles and crusting. The face, palms and soles are often spared. Healing occurs in one to two months with supple, reticulated scarring.

“There is some worry though because the child could experience hypohidrosis/heat intolerance, scarring alopecia later in life,” Dr. Shaffer explains. “There also could be developmental delay in some with a question as to if it is related to CEVD or prematurity.”

• Mastocytoma may present in solitary form or a few and there are often blisters in neonates and infants.

“There is no need for worry with mastocytoma, because the blistering subsides in the first few years of life,” Dr. Shaffer says. “The lesions tend to resolve spontaneously during childhood.”

• Diffuse cutaneous mastocytosis is characterized by leathery skin with hiving, flushing and often blistering.

“There is some worry because high mast cell ‘load’ puts at risk for hypotension and respiratory distress related to mediator release,” Dr. Shaffer says. “The child many have symptoms from GI or CNS involvement, but it often resolves or improves substantially during childhood.”