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[Multicenter histiocytosis]
A M Schmitt1, P Grolleau-Rochiccioli, G Laplanche
1Service de Dermatologie, CHR Purpan, Toulouse.
Introduction:
Multicentric histiocytosis is a rare systemic disease with active episodes and prolonged periods of remission. Immunosuppressor treatment (alkylating agents) can be effective.
Case Report:
A new case of multicentric histiocytosis was observed in a 38-year-old man who was successfully treated with cyclophosphamide at the dose of 100 mg/d. Treatment was withdrawn after several weeks due to drug-induced hepatitis and replaced with chlorambucil at the dose of 0.1 mg/kg/day for 6 months. No relapse has occurred after a follow-up of 14 months.
Discussion:
This case is particularly interesting because of the exceptional nature of spontaneous haemarthrosis which was the inaugural sign of joint manifestations with mediastinal lymph nodes and initially isolated pruritus occurring before the typical skin manifestations. Different management protocols have been discussed with emphasis on the presence of acute cyclophosphamide induced hepatitis. This immunosuppressor is not usually hepatotoxic.
Conclusion:
This case demonstrates the systemic nature of the disease and emphasizes the beneficial effect of alkylating agents.