A Case of Cytophagic Histiocytic Panniculitis Involving the Extrapleural Fat.
- Author:
Jong Keun SEO
1
;
Joon Hee CHOI
;
Sang Min LEE
;
Sook Kyung LEE
Author Information
1. Department of Dermatology, Maryknoll Hospital, Busan. Korea. karrot75@hanmail.net
- Publication Type:Case Report
- Keywords:
Cytophagic histiocytic panniculitis;
Extrapleural fat
- MeSH:
Cytophagocytosis;
Drug Therapy, Combination;
Fever;
Histiocytes;
Humans;
Lymphoproliferative Disorders;
Panniculitis*;
Serositis;
Skin;
Subcutaneous Tissue;
T-Lymphocytes
- From:Korean Journal of Dermatology
2007;45(2):180-184
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Cytophagic histiocytic panniculitis gap (CHP) was described in 1980 as a chronic histiocytic disease of the subcutaneous tissue and associated with systemic manifestations such as fever, serositis, and hepatosplenomegaly. The current concept of CHP is that it represents a spectrum of lymphoproliferative disorders that induce secondary histiocytic cytophagocytosis. The pathogenesis of hemophagocytosis in CHP is unknown but may be related to histiocyte reaction to an abnormal cytokine milieu brought about by disordered T-cell function. In most cases the disease follows a fulminant course, but in some patients the disease seems limited to the skin and subcutaneous tissue and follows a more benign, chronic course. We report a case of cytophagic histiocytic panniculitis which had a long chronic course over 16 years, but recently became aggravated with the involvement of the extrapleural fat tissue and was treated with combination chemotherapy.
