Eruptive xanthoma-like cutaneous Langerhans cell histiocytosis in an adult

Abstract

A 45-year-old man had a 1-year history of asymptomatic papules on his face, neck, trtmk, and extremities. Examination revealed multiple, discrete, yellow-red papules (Fig. 1). Histopathologic examination of a papule from the left ann showed a dense infiltrate ofmononuclear cells in the papillary and reticular dermis. There were many histiocytic ceils with an abundant, pale cytoplasm and an eccentric, indented nucleus (Fig. 2). Numerous histiocytic cells with a kidney-shaped nucleus and a few clear cells were also observed. Touton giant cells were absent. Giemsa stain showed occasional mast cells. Staining with Oil Red O was negative. Most of the histiocytic ceils stained positive for S-100 protein and CDla. Ultrastructural study revealed Birbeck granules in the cytoplasm of the dermal histiocytic cells. The results of the following studies were within normal limits or negative; blood cell count, liver function tests, coagulation studies, immunoglobulins, and urinalysis. The fasting serum lipid profile was also normal. Findings of roentgenograms taken of the skull and chest were normal. Bone scan as well as computed tomographic scanning of the brain, chest, abdomen, and pelvis revealed no evidence of internal organ involvement. We concluded that this was LCH confined to the skin. Treatment was started with topical carmustine and PUVA. Four weeks later, the patient's condition had improved considerably, and 6 months later there was no evidence of recurrence.