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A Case of Cytophagic Histiocytic Panniculitis Associated with Hemophagocytic Lymphohistiocytosis

Hyunji Lee, Ham Ji-Yeon, Park Kyung-Duck

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Abstract

Cytophagic histiocytic panniculitis (CHP) is a rare form of panniculitis, presenting lymphohistiocytic infiltration within subcutaneous fat tissue with phagocytic histiocytes. Associated systemic symptoms includes fever, hepatosplenomegaly, lymphadenopathy, serositis, pancytopenia, hepatic abnormalities, hypertriglyceridemia, and coagulopathy, which are the features of hemophagocytic lymphohistiocytosis (HLH). The patients of CHP associated with HLH may have nonfatal acute/intermittent, or rapidly fatal clinical courses, so the prompt and accurate diagnosis with immunosuppressive treatments are significant. (Korean J Dermatol 2021;59(2):157∼159)

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What this paper is about

Cytophagic histiocytic panniculitis (CHP) is a rare form of panniculitis, presenting lymphohistiocytic infiltration within subcutaneous fat tissue with phagocytic histiocytes. Associated systemic symptoms includes fever, hepatosplenomegaly, lymphadenopathy, serositis, pancytopenia, hepatic abnormalities, hypertriglyceridemia, and coagulopathy, which are the features of hemophagocytic lymphohistiocytosis (HLH). The patients of CHP associated with HLH may have nonfatal acute/intermittent, or rapidly fatal clinical courses, so the prompt and accurate diagnosis with immunosuppressive treatments are significant. (Korean J Dermatol 2021;59(2):157∼159)

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Available abstract

Cytophagic histiocytic panniculitis (CHP) is a rare form of panniculitis, presenting lymphohistiocytic infiltration within subcutaneous fat tissue with phagocytic histiocytes. Associated systemic symptoms includes fever, hepatosplenomegaly, lymphadenopathy, serositis, pancytopenia, hepatic abnormalities, hypertriglyceridemia, and coagulopathy, which are the features of hemophagocytic lymphohistiocytosis (HLH). The patients of CHP associated with HLH may have nonfatal acute/intermittent, or rapidly fatal clinical courses, so the prompt and accurate diagnosis with immunosuppressive treatments are significant. (Korean J Dermatol 2021;59(2):157∼159)

Key concepts: Hemophagocytosis, Hemophagocytic lymphohistiocytosis, Medicine, Pancytopenia, Hepatosplenomegaly, Panniculitis, Histiocyte, Coagulopathy

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