Cutaneous Langerhans Cell Histiocytosis as Presenting Sign of Systemic B-Cell Lymphoma.

Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disorder most commonly involving the bone and skin; however, it can arise in many other locations. It is predominantly a pediatric disease, but adult cases occur. Here, we present a case of adult-onset cutaneous LCH with systemic symptoms, believed to represent disseminated LCH. Further evaluation, however, revealed concomitant bone marrow involvement by a small B-cell lymphoma. An association between B-cell lymphoma and cutaneous LCH has only rarely been previously reported. This report adds to the growing body of literature, however, on associations of cutaneous LCH with hematologic malignancies, and it illustrates the need for a complete systemic evaluation including a bone marrow biopsy in suspected cases of disseminated LCH.