Single System Cutaneous Langerhans Cell Histiocytosis Mimicking Prurigo Nodularis / 대한피부과학회지

ABSTRACT

Langerhans cell histiocytosis (LCH) is a rare inflammatory neoplasia of the dendritic cells that can affect the whole body and commonly affect the pediatric age. Adult onset LCH is relatively rare, and the cutaneous form without any other organ involvement is much more uncommon. A 62-year-old woman visited the clinic presenting pruritic erythematous papule and nodules on the whole body, from which she suffered for several years. Skin biopsy showed hyperkeratosis, mild acanthosis, intra-epidermal tumor cell infiltration, and band-like tumor cell infiltration. Cells infiltrating the epidermis and dermis showed characteristics consistent with Langerhans cells. Immunohistochemical analysis revealed tumor cells highlighted with CD1a and S-100, but not with CD68, and therefore, diagnosed as LCH. The patient showed no evidence of any other organ manifestation, which led the final diagnosis of single-system LCH. This case highlights a rare adult-onset cutaneous form of single-system LCH with skin manifestations very similar to prurigo nodularis.