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[Histiocytic cytophagic panniculitis]
1Clinique dermatologique des Hôpitaux Universitaires de Strasbourg.
Presse Medicale (Paris, France : 1983)
|April 16, 1998
Summary
Histiocytic cytophagic panniculitis, a skin manifestation of reactive hemophagocytic syndrome, involves benign histiocytic infiltration of fat. This condition, often triggered by infections in immunocompromised individuals, requires prompt diagnosis and treatment.
Area of Science:
- Dermatology
- Hematology
- Immunology
Context:
- Histiocytic cytophagic panniculitis (HCP) is a specific skin finding in reactive hemophagocytic syndrome (RHS).
- HCP presents as a febrile nodular eruption, accompanying other RHS clinical and biological features.
- Histopathologic examination reveals lobular panniculitis with benign histiocytic infiltration and variable phagocytosis.
Purpose:
- To detail the histopathological diagnosis of HCP.
- To explore the triggers and associations of HCP, including infections and T-cell lymphomas.
- To discuss the pathophysiology involving abnormal cytokine secretion from T cells.
Summary:
- HCP is characterized by histiocytic infiltration of subcutaneous fat.
- Infections and altered immune function frequently trigger HCP, necessitating T-cell lymphoma screening.
- Abnormal cytokine secretion by T cells activates histiocytes in HCP.
Impact:
- Untreated HCP typically follows a lethal course.
- Treatment options include addressing the etiology, symptomatic management (transfusions, corticosteroids), chemotherapy (etoposide, cyclosporine, CHOP-like regimens), and intravenous immunoglobulin.
- Early diagnosis and appropriate treatment are crucial for patient outcomes.