[Panniculitis with cellular phagocytosis. Chronic form of histiocytic panniculitis with fever, pancytopenia, polyserositis and lethal hemorrhagic diathesis].
Two cases of what was originally called "Weber-Christian disease" were found to be a chronic, recurrent form of histiocytic panniculitis characterized by histiocytic engulfment of red and white blood cells--"cytophagocytosis." The disease was manifested by recurrent fever, subcutaneous nodules, pancytopenia, and mucosal ulcerations. Hepatosplenomegaly and enlarged lymph nodes were observed. Focal invasion of the reticuloendothelial system by acidophilic or cytophagic histiocytes could be identified. Progressive changes in liver function and hemorrhagic diathesis occurred in both patients.
D000273:Adipose Tissue / Q000473:pathology; D000328:Adult; D001853:Bone Marrow / Q000473:pathology; D002908:Chronic Disease; D005260:Female; D005334:Fever / Q000209:etiology; D006474:Hemorrhagic Disorders / Q000209:etiology; D006644:Histiocytes / Q000502:physiology / Q000648:ultrastructure; D006801:Humans; D012157:Mononuclear Phagocyte System / Q000473:pathology; D010198:Pancytopenia / Q000209:etiology; D010201:Panniculitis, Nodular Nonsuppurative / Q000175:diagnosis* / Q000473:pathology; D010587:Phagocytosis; D012867:Skin / Q000473:pathology
Pannikulitis mit Zellphagozytose. Eigenständige Form einer histiozytären Pannikulitis mit Fieber, Panzytopenie, Polyserositis und letaler hämorrhagischer Diathese.