PMID: 9537003Apr 16, 1998Paper

Histiocytic cytophagic panniculitis

La Presse médicale
D Lipsker, E Grosshans

Abstract

A HISTOPATHOLOGICAL DIAGNOSIS: Histiocytic cytophagic panniculitis is the specific skin finding in the reactive hemophagocytic syndrome. It is a feverish nodular eruption which accompanies the other clinical and biological features of the reactive hemophagocytic syndrome. Histopathologic changes are diagnostic and consist in a lobular panniculitis characterized by the presence of a histiocytic infiltration of the fat. Histiocytes are benign in appearance and show variable degree of phagocytosis. In more than 50% of the cases, the disease is triggered by an infection (mainly a viral infection from the herpes-virus family) in a patient with altered immune function (immunodeficiency, autoimmune disease, hematological disease). Search for subcutaneous T-cell lymphoma is mandatory, but is a diagnostic challenge. Such lymphomas are associated with histologic features of histiocytic cytophagic panniculitis. Histiocytes in the histiocytic cytophagic panniculitis are activated by an abnormal cytokine secretion (interferon gamma, granulocyte/macrophage colony stimulating factor, phagocytosis inducing factor, ...) from neoplastic or reactive T cells. Untreated, the course of the disease is usually lethal. Etiologic treatment should be pro...Continue Reading

Citations

Apr 20, 2000·The Annals of Pharmacotherapy·L E BoccuminiK H Kaidbey

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