Cytophagic histiocytic panniculitis. Systemic histiocytosis presenting as chronic, nonhealing, ulcerative skin lesions.
Systemic histiocytosis was found in a patient who presented with chronic, nodular, and ulcerative skin lesions. The patient's hospital course was complicated by persistent fever, thrombocytopenia, severe neutropenia, and coagulation abnormalities. Treatment consisted only of splenectomy and supportive care. Postoperatively, the patient's skin lesions and the fever, pancytopenia, and coagulopathy resolved. Marked proliferation of histologically benign macrophages was observed in dermal, splenic, hepatic, and lymphoid tissues; leukophagocytosis and erythrophagocytosis were clearly demonstrated. This illness is most consistent with cytophagic histiocytic panniculitis, a newly described syndrome.
D000273:Adipose Tissue / Q000473:pathology; D000328:Adult; D002908:Chronic Disease; D006801:Humans; D008206:Lymphatic Diseases / Q000473:pathology*; D008264:Macrophages / Q000502:physiology; D008297:Male; D010201:Panniculitis, Nodular Nonsuppurative / Q000473:pathology*; D010587:Phagocytosis; D012867:Skin / Q000473:pathology; D012883:Skin Ulcer / Q000473:pathology*; D013154:Spleen / Q000473:pathology