A 37-year-old male with systemic lupus erythematosus (SLE) presented with high fever, subcutaneous indurations, anemia, thrombocytopenia, elevated liver enzymes and hyperferritinemia. Skin biopsy revealed hemophagocytic histiocytes in the adipose tissues. The patient was diagnosed with SLE with cytophagic histiocytic panniculitis (CHP). Treatment with high-dose glucocorticoids and cyclosporine A induced remission of SLE and CHP.
CHP is generally a systemic disorder affecting subcutaneous adipose tissues with a high mortality rate. However, based on the present and previously reported cases, we believe that intensive immunosuppression can ameliorate CHP that occurs as a skin manifestation of SLE.
MoriYSugiyamaTChibaR. A case of systemic lupus erythematosus with hemophagocytic syndrome and cytophagic histiocytic panniculitis. Rheumachi2001; 41: 31–36.
6.
OstrovBEAthreyaBHEichenfieldAHGoldsmithDP. Successful treatment of severe cytophagic histiocytic panniculitis with cyclosporine A. Semin Arthritis Rheum1996; 25: 404–413.
7.
MiyabeYMurataYBabaYItoENagasakaK. Successful treatment of cyclosporine-A-resistant cytophagic histiocytic panniculitis with tacrolimus. Mod Rheumatol2011; 21: 553–556.
8.
WhiteJWJrWinklemannRK. Cytophagic histiocytic panniculitis is not always fatal. J Cutan Pathol1989; 16: 137–144.
9.
JaffeECostaJFauchiASCossmanJTsokosM. Malignant lymphoma and erythrophagocytosis simulating malignant histiocytosis. Am J Med1983; 75: 741–749.
10.
LayJTsaoCChenJKadinMESuI. Upregulation of tumor necrosis factor-a gene by Epstein-Barr virus and activation of macrophages in Epstein-Barr virus-infected T cells in the pathogenesis of hemophagocytic syndrome. J Clin Invest1997; 100: 1969–1979.
11.
KasaharaYYachieA. Cell type specific infection of Epstein-Barr virus (EBV) in EBV-associated hemophagocytic lymphohistiocytosis and chronic active EBV infection. Crit Rev Oncol Hematol2002; 44: 283–294.
12.
RequenaLYusES. Panniculitis. Part II. Mostly lobular panniculitis. J Am Acad Dermatol2001; 45: 325–361.
