Necrotic, ulcerated papules on a newborn male

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A 1-day-old Hispanic boy presented to our department with truncal lesions that had been present since birth. The infant was born at term without prenatal or perinatal complications and was otherwise healthy. The physical examination revealed an afebrile patient with three 5-mm eroded papules with black eschars—one each on the right posterior axilla, left posterior axilla, and right buttock (Fig 3). Two 4-mm punch biopsy specimens were taken from two of the lesions. Histologic examinations of the ulcerated skin revealed an infiltrate of large, pleomorphic cells with oval to indented nuclei admixed with some neutrophils, eosinophils, and lymphocytes. Mitoses were readily identified (Fig 4). The infiltrate was heaviest in the papillary dermis, with focal epidermotropism and extension into the reticular dermis. Immunohistochemical stains were strongly positive for S-100 protein (Fig 5) and CD1a in the papillary dermal proliferation. 6.What is the most likely diagnosis? a.Congenital candidiasis b.Neonatal disseminated hemangiomatosis c.Langerhans cell histiocytosis d.Congenital leukemia e.Neonatal pustular melanosis 7.Immunohistochemical stains of this condition show that the cells demonstrate which of the following? a.S-100 b.CD1a c.Interferon-γ receptor d.Placental alkaline phosphatase e.All of the above 8.A higher degree of which of the following findings suggests a self-healing process as opposed to a multisystem disease? a.Necrosis b.Ulceration c.Dense infiltrate of eosinophils d.All of the above e.None of the above 9.Treatment of this condition when limited to the skin includes which of the following? a.Systemic chemotherapy b.Immediate excision of the lesions c.Long-term follow-up d.All of the above e.None of the above View Large Image Figure Viewer Download Hi-res image View Large Image Figure Viewer Download Hi-res image