When a newborn rash is not benign

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ESPR234
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History: 

A female infant was born vaginally to a 30 year-old gravida 5 para 4 mother. Maternal history included remote intravenous drug use and herpes simplex virus type 2. Maternal urine toxicology was positive for methadone. Maternal labs were blood type O+, antibody negative, Hepatitis B and C negative, Rubella immune, HIV negative, Gonorrhea and Chlamydia negative, varicella nonimmune. Infant was assigned APGAR scores of 9 and 9 at 1 and 5 minutes respectively. Infant received standard newborn care after birth and was transferred to Neonatal Intensive Care Unit (NICU) for evaluation of congenital skin rash.


Physical exam:  

At time of birth, the exam was notable for multiple ulcerations in various stages of healing, most notably 1cm lesion on left occiput, left elbow, left thigh fold and between 4th and 5th toes on right foot along with 3 small ulcerations on back. On the next day, two new erythematous plaques were noted on left shoulder. 


After transfer to the NICU, the infant had evolving rashes on chin with pink papules and macules. New pinpoint hemorrhagic papules were noted on lower lip and bilateral cheeks. On the crown of the scalp, left elbow, right chest, right arm, upper back, and left hand there were scattered hemorrhagic crusted papules and plaques on an erythematous base. Some were slightly punched out or with a scalloped border. There were ruptured red-brown bullae on the neck. Nikolsky sign was negative, and there was no mucosal involvement noted on examination. 


The remainder of the examination was within normal limits. 


Laboratory or Diagnostic imaging or Procedures:

The infant received a thorough infectious workup including varicella PCR and herpes simplex virus 1/2 surface PCR that were both negative. Blood HSV PCR was found to be negative. Surface cultures of the lesion were obtained for aerobic, anaerobic and fungal cultures that were negative. Serum electrolytes, liver function tests, and a CBC with differential were normal. Subsequently a diagnostic procedure was done which revealed the eventual diagnosis.  


Final diagnosis:

A punch biopsy was performed which revealed a presence of focal aggregates of CD1a cells in the papillary dermis, dermal infiltrates of Langerhans cells with indented nuclei and pale eosinophilic cytoplasm, and admixed inflammatory cells. The Langerhans cells also involved the overlying hyperplastic epidermis. These findings were consistent with a diagnosis of Langerhans Cell Histiocytosis.


Discussion


The vast majority of newborn skin rashes are benign. Langerhans Cell Histiocytosis (LCH) is a rare disease with estimated incidence of three to five cases per million, with newborn cases estimated at one to two cases per million2. It is more commonly diagnosed in children than adults. LCH should be suspected when there are ulcerative, purpuric scattered lesions present on the newborn skin in various stages of eruption and healing. It is also classically diagnosed in older infants with persistent seborrheic dermatitis and diaper dermatitis that are unresponsive to traditional treatments. 

Histiocytes are mononuclear precursors of antigen-presenting cells, and LCH is a proliferation of the Langerhans cells, dendritic antigen presenting cells found in lymph nodes and skin. Diagnosis of LCH is by skin biopsy, showing positive immunohistochemical staining for Cd1a in mononuclear cells in the dermis and S100 in the epidermis. 

There are four known clinical forms of LCH: Letterer-Siwe (acute disseminated disease), Hand-Schuller-Christian (multifocal chronic disease), focal or eosinophilic granuloma, and Hashimoto Pritzker disease (congenital, self-limiting localized skin lesions). The skin is affected in 50% of cases and is the only organ effected in 10% of cases. Although the disease can present congenitally with localized skin findings, rare case reports suggest that over time it can progress to multiorgan disease. Multiorgan involvement can be seen in the liver, spleen, lungs, and bone marrow. If multisystemic disease exists, treatment is with chemotherapy in one or two cycles, depending on the level of organ involvement. In rare cases, focal skin lesions can progress to multifocal disease, and eventually to death1; therefore, close follow up with pediatric oncology is crucial, even if there is remission of skin lesions. 


In this described case, a thorough workup to evaluate other organ involvement was pursued, including urinalysis, urine electrolytes, skeletal survey, liver function tests, Hgb electrophoresis which was normal and thus this case was diagnosed as Hashimoto Pritzker subtype. Patient will have close follow up with hematology/oncology and dermatology to monitor for further symptoms. 

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Tufts Children's Hospital
Tufts Children's Hospital at Tufts Medical Center
Tufts Children's Hospital

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