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


1, 2, 3, 4, 5, 6, 7

 

  1. Infectious Disease Clinic, University Hospital Santa Maria della Misericordia, Udine, Italy.
  2. Rheumatology Clinic, Department of Medical and Biological Sciences, University Hospital Santa Maria della Misericordia, Udine, Italy.
  3. Infectious Disease Clinic, University Hospital Santa Maria della Misericordia, Udine, Italy. mattba@tin.it
  4. Infectious Disease Clinic, University Hospital Santa Maria della Misericordia, Udine, Italy.
  5. Rheumatology Clinic, Department of Medical and Biological Sciences, University Hospital Santa Maria della Misericordia, Udine, Italy.
  6. Institute of Pathology, University Hospital Santa Maria della Misericordia, Udine, Italy.
  7. Rheumatology Clinic, Department of Medical and Biological Sciences, University Hospital Santa Maria della Misericordia, Udine, Italy.

CER8522
2015 Vol.33, N°6
PI 0906, PF 0909
Case Reports

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PMID: 26575530 [PubMed]

Received: 14/04/2015
Accepted : 13/07/2015
In Press: 17/11/2015
Published: 15/12/2015

Abstract

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.

Rheumatology Article