Successful treatment of cyclosporine-A-resistant cytophagic histiocytic panniculitis with tacrolimus

Abstract

A 34-year-old woman with discoid lupus erythematosus and lupus profundus was admitted to our hospital showing signs of a fever, malaise, and abdominal swelling. Diagnosis of cytophagic histiocytic panniculitis (CHP) was made based on lobular panniculitis with a hemophagocytosis. Treatment with high doses of prednisolone combined with cyclosporine A (CsA) was not effective enough. However, after changing CsA to tacrolimus (TAC), CHP improved. Our case demonstrates that TAC may be a novel therapy for CHP.

Systemic lupus erythematosus with cytophagic histiocytic panniculitis successfully treated with high-dose glucocorticoids and cyclosporine A

“…Actually, multiple cases of life-threatening CHP successfully treated with cyclosporine A or tacrolimus are reported. 6,7 Single cases of CHP controlled with azathioprine, anakinra or cyclophosphamide with intravenous immunogloblins are reported as well. 2,5 These agents could be considered as a salvage therapy for CHP resistant to cyclosporine A or tacrolimus.…”

“…1,6 While cyclosporine A administration was reported to be an effective treatment for life-threatening CHP, 6 CHP resistant to cyclosporine A is reported. 2,5,7 Thus, CHP still remains a fatal disorder.…”

“…Additionally, all four previously reported cases of SLE and DLE with CHP accompanying poor prognostic factors were treated successfully with immunosuppressants. [3][4][5]7 In contrast to cases of malignant lymphoma, EBV infection and unknown causes, CHP can be treated successfully by intensive immunosuppression when it is associated with SLE. 1,2 Although the pathogenesis of CHP is still unclear, it is assumed that cytokines secreted from aberrantly immunoregulated T cells activate macrophages to become cytophagic histiocytes.…”

Paniculitis histiocítica citofágica con remisión espontánea

“…El manejo de la PHC en general se basa en el uso de inmunosupresores, inmunomoduladores y citotóxicos cuyo blanco sean los macrófagos e histiocitos activados (corticosteroides, etopósido, dosis altas de inmunoglobulina intravenosa) y los linfocitos T activados (corticosteroides, ciclosporina) 7,8 . Se publicaron casos de manejo exitoso con tacrolimús y anakinra 9,10 . Las formas clínicas más agresivas presentaron una mortalidad del 70%, aun en aquellos casos en los que el tratamiento era el indicado 9 .…”

“…Se publicaron casos de manejo exitoso con tacrolimús y anakinra 9,10 . Las formas clínicas más agresivas presentaron una mortalidad del 70%, aun en aquellos casos en los que el tratamiento era el indicado 9 .…”