FATAL CYTOPHAGIC HISTIOCYTIC PANNICULITIS AFTER A SHORT RESPONSE TO CYCLOSPORINE

Cytophagic histiocytic panniculitis (CHP) is a disorder characterized by a histiocytic infiltrate in the subcutaneous adipose tissue which o ften extends to involve systemic organs. Fever, pancytopenia, hepatosp lenomegaly, mucosal ulcers and serositis are common systemic symptoms. Although remissions have been reported, the disease tends to follow a chronic course often complicated by terminal hemorrhagic diathesis an d death. Several reports in the recent literature have suggested that cyclosporine is the treatment of choice in CHP. We report the first ca re of cyclosporine treatment failure in CPH. Despite an initial respon se to treatment and persistent resolution of cutaneous lesions, the in ternal disease progressed resulting in the patient's death. Therefore the evolution of skin lesions and signs of systemic involvement may no t be an accurate assessment of disease activity in patients with CHP b eing treated with cyclosporine. (C) 1998 Elsevier Science B.V.