Cytophagic histiocytic panniculitis with fever, cytopenia, liver failure, and terminal hemorrhagic diathesis.
We have seen five adult patients with a clinical picture of recurrent histiocytic, cytophagic panniculitis, cytopenia, abnormal liver function tests, and a terminal, febrile bleeding diathesis. Originally thought to have Weber-Christian disease, these patients, we believe, represent a unique syndrome: lobular, histiocytic, cytophagic panniculitis. Erythrophagocytosis and cytophagocytosis are readily observed, but the cells do not show malignant features. Histiocytosis can be found at times in the bone marrow, lymph nodes, liver, spleen, and serosal tissues, as well as in the skin and subcutaneous tissues. The terminal hemorrhage in these patients is characterized by features of pancytopenia, liver failure, and intravascular coagulation. This disease may be separated from malignant histiocytosis by the chronic course, the primary involvement of the adipose tissue, and the benign histiocytes in the infiltrate. It has some similarities to other regional histiocytoses such as sinus histiocytosis, intestinal histiocytosis, and splenic histiocytosis.
D000328:Adult; D000368:Aged; D005260:Female; D005334:Fever / Q000209:etiology; D006474:Hemorrhagic Disorders / Q000209:etiology*; D006644:Histiocytes / Q000473:pathology; D006801:Humans; D008107:Liver Diseases / Q000209:etiology*; D008198:Lymph Nodes / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010198:Pancytopenia / Q000209:etiology*; D010201:Panniculitis, Nodular Nonsuppurative / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D010587:Phagocytosis; D012008:Recurrence; D013577:Syndrome