Langerhans Cells Histiocytosis in One Family

Abstract

Histiocytosis of Langerhans cells (class 1 histiocytosis) includes a range of clinical manifestations that have been described as bone eosinophilic granuloma, Hand–Schüller–Christian syndrome, and Letterer–Siwe diseases. This syndrome represents a spectrum of severity and prognosis of some underlying disorder which is usually sporadic. This report discribes three cases in one family, who developed the disease a few years after their brother was found to be suffering from histiocytosis. All 3 patients had the same clinical manifestations: hyperthermia, eczematic rash, and swelling in skull, hand, and foot. X rays showed lytic areas in the skull and metacarp of fourth finger. Serology for EBV infection was negative. Infiltratration of abnormal Langerhans cells histiocytes were demonstrated in bone biopsies. These patients were given chemotherapy. Case 1 (brother) died 1 year after chemotherapy, case 2 (girl) was given chemotherapy without success. She was given T-cell suppresor (cyclosporine), which induced remission, and case 3 was given chemotherapy, which was successful.

• eosinophilic granuloma
• familial histiocytosis
• histiocytosis X
• Langerhans cell (LCH)
• Letterer–Siwe disease