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 often extends to involve systemic organs. Fever, pancytopenia, hepatosplenomegaly, 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 and death. Several reports in the recent literature have suggested that cyclosporine is the treatment of choice in CHP. We report the first case of cyclosporine treatment failure in CPH. Despite an initial response to treatment and persistent resolution of cutaneous lesions, the internal disease progressed resulting in the patient's death. Therefore the evolution of skin lesions and signs of systemic involvement may not be an accurate assessment of disease activity in patients with CHP being treated with cyclosporine.
Guitart
J
J
Department of Dermatology, Northwestern University Medical Center, Chicago, IL 60611, USA.
Sethi
R
R
Gordon
K
K
D016572:Cyclosporine / Q000627:therapeutic use*; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D005260:Female; D015614:Histiocytosis / Q000175:diagnosis* / Q000188:drug therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008875:Middle Aged; D015434:Panniculitis / Q000175:diagnosis* / Q000188:drug therapy*
D007166:Immunosuppressive Agents; D016572:Cyclosporine