The cutaneous manifestations of sinus histiocytosis with massive lymphadenopathy

Abstract

Ten patients with sinus histiocytosis with massive lymphadenopathy (SHML) also had cutaneous involvement. Seven of the 10 were children. The skin lesions were solitary in 3 patients and multiple in 7. They were papular or nodular, up to 4 cm in diameter and often had a xanthomatous appearance. Microscopically, they were constituted by a dermal infilitrate made up predominantly of histiocytes, plasma cells and lymphocytes. Some of the histiocytes contained phagocytosed lymphocytes in their cytoplasm. The microscopic differential diagnosis includes dermatofibroma, xanthoma, Tangier disease, histiocytosis X, reticulohistiocytoma, juvenile xanthogranuloma and leprosy.