Efficacy of intravenous cyclosporine in a case of cytophagic histiocytic panniculitis complicated by haemophagocytic syndrome after visceral leishmania infection

Clin Exp Rheumatol. 2015 Nov-Dec;33(6):906-9. Epub 2015 Nov 17.


Cytophagic histiocytic panniculitis (CHP) is a rare panniculitis characterized by systemic features, due to histiocytic infiltration along with haemophagocytosis, which may also appear in bone marrow, spleen, lymph nodes, and liver. Haemophagocytic lymphohistiocytosis (HLH) is a group of autoinflammatory disorders, which include macrophage activation syndrome, sometimes observed in the course of systemic autoimmune diseases, such as juvenile chronic polyarthritis, systemic lupus erythematosus or vasculitis, and infection-associated haemophagocytic syndrome; if not promptly recognised and treated, HLH can be fatal. Visceral leishmaniasis (VL) is a systemic disease caused by different forms of Leishmania spp., an intracellular protozoa. VL is endemic in tropical countries such as in the Middle East and the Mediterranean. The typical clinical and laboratory features are fever, hepato-splenomegaly, hypergammaglobulinaemia and pancytopenia. The features of VL may mimic some haematologic diseases. We report a case of cytophagic histiocytic panniculitis and HLH, triggered by a previous visceral leishmania infection. Cyclosporine was quickly effective in this case, after failure of high-dose glucocorticoids, anakinra and etoposide.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antirheumatic Agents / administration & dosage
  • Antirheumatic Agents / adverse effects
  • Bone Marrow / parasitology
  • Bone Marrow Examination / methods
  • Cyclosporine* / administration & dosage
  • Cyclosporine* / adverse effects
  • Drug Monitoring
  • Drug Substitution / methods
  • Glucocorticoids / administration & dosage
  • Glucocorticoids / adverse effects
  • Histiocytosis* / diagnosis
  • Histiocytosis* / etiology
  • Humans
  • Interleukin 1 Receptor Antagonist Protein / administration & dosage
  • Interleukin 1 Receptor Antagonist Protein / adverse effects
  • Leishmania* / drug effects
  • Leishmania* / isolation & purification
  • Leishmaniasis, Visceral* / complications
  • Leishmaniasis, Visceral* / diagnosis
  • Leishmaniasis, Visceral* / drug therapy
  • Leishmaniasis, Visceral* / physiopathology
  • Lymphohistiocytosis, Hemophagocytic* / diagnosis
  • Lymphohistiocytosis, Hemophagocytic* / drug therapy
  • Lymphohistiocytosis, Hemophagocytic* / etiology
  • Lymphohistiocytosis, Hemophagocytic* / physiopathology
  • Male
  • Panniculitis* / diagnosis
  • Panniculitis* / etiology
  • Treatment Outcome


  • Antirheumatic Agents
  • Glucocorticoids
  • Interleukin 1 Receptor Antagonist Protein
  • Cyclosporine