A CASE OF CUTANEOUS LANGERHANS CELL GRANULOMATOSIS SUCCESSFULLY TREATED WITH 2-CHLORODEOXYADENOSINE AND THALIDOMIDE

We describe the case of a 65-year-old female patient with cutaneous Langerhans' cell granulomatosis without any signs of disease in other organs. She also had systemic lupus erythematosus that had been diagnosed several years before. The coexistence of these two diseases has not been described before as far as we know. The purine analogue 2-chlorodeoxyadenosine (Cladribin), which has been used successfully in the treatment of hairy cell leukaemia, induced complete remission in our patient after 1 week of treatment. After 2 months, however, the patient had a relapse; this was successfully treated with thalidomide. A new understanding of Langerhans' cell granulomatosis as a reactive but not cancerous disease has emerged as a result of recent investigations showing that tumour necrosis factor-alpha (TNF-alpha) plays an important part in the induction of Langerhans' cells from their immature precursors. Because thalidomide has been shown to inhibit TNF-alpha production, down-modulation of this cytokine seems to be a useful treatment strategy in Langerhans' cell granulomatosis. Some aspects of the diagnosis and therapy of this disease are briefly reviewed.