Cytophagic histiocytic panniculitis is not always fatal

Abstract

Two patients with benign cytophagic histiocytic panniculitis are presented and compared with the one benign and ten fatal cases previously reported. Benign-appearing histiocytes which engulf blood cells, nuclear debris, and platelets are the most characteristic feature of the disease. This cytophagia tends to be massive in the subcutaneous tissue and involves extracutaneous organs only in the fatal cases. The course is chronic in the benign form. Patients with the fatal form tend to have fever, hepatosplenomegaly, serosal effusions, ecchymoses, peripheral adenopathy, and mucosal ulcers. Anemia, leukopenia, elevated liver enzyme levels, and coagulopathy are present in almost all and hypocalcemia in many patients with a fatal course. Cytophagic histiocytic panniculitis may represent a form of regional histiocytosis primarily involving subcutaneous tissue. It is midway in the spectrum of the cytophagic histiocytoses.