Successful treatment of severe cytophagic histiocyticpanniculitis with cyclosporine A

Ostrov, Barbara E.; Athreya, Balu H.; Eichenfield, Andrew H.; Goldsmith, Donald P.

doi:10.1016/s0049-0172(96)80005-9

Cited by 43 publications

(36 citation statements)

References 44 publications

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“…It suggests that CSA is the treatment of choice for nonmalignant severe CHP associated with MAS. 14,15 These features and the immunochemical pattern of in situ MAS in the panniculitis lesions emphasize the key role of T cells in the pathogenesis of CHP.…”

Section: Figurementioning

confidence: 94%

Clonal Cytophagic Histiocytic Panniculitis in Children May Be Cured by Cyclosporine A

Bader-Meunier

Fraïtag

Janssen³

et al. 2013

Pediatrics

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Cytophagic histiocytic panniculitis (CHP) is a rare panniculitis in childhood, associated either with nonmalignant conditions or with subcutaneous panniculitis-like T-cell lymphoma (SPTCL), and often also associated with macrophage activation syndrome (MAS). Discriminating between these 2 conditions is therapeutically important because nonmalignant CHP often improves under cyclosporine and prednisone, whereas most cases of SPLT may be best treated with more aggressive therapy. We report the cases of a 6-month-old boy and a 16-month-old girl who, after viral infection, developed multiple infiltrating skin nodules on the limbs and face, associated with MAS. Histopathologic findings for skin biopsy specimens revealed CHP associated with heavily cellular lobular panniculitis. Hemophagocytosis and immunohistochemical staining features were consistent with typical characteristics of in situ MAS in adipose tissue: the lymphocytes were mostly TCD8+ cells with an activated phenotype (human leukocyte antigen (HLA) -DR+) and expressed interferon-g; CD68+ macrophages expressed tumor necrosis factor-a and interleukin-6. A monoclonal rearrangement of the T-cell receptor g gene was present in skin tissue but not in peripheral blood or bone marrow lymphocytes. Cyclosporine A treatment resulted in the complete remission of cutaneous and systemic manifestations in both patients for 66 and 29 months, respectively. This report suggests that the diagnosis of a reactive T-cell lymphoproliferation should be the treatment of choice in young children with severe CHP, even if there is a SPTCL-like aspect with an in situ T-cell clonality. It also suggests that CSA is the optimal treatment of this condition and postulates the possible pathologic process underlying this efficacy. Pediatrics 2013;132:e545-e549

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Section: Figurementioning

confidence: 94%

Clonal Cytophagic Histiocytic Panniculitis in Children May Be Cured by Cyclosporine A

Bader-Meunier

Fraïtag

Janssen³

et al. 2013

Pediatrics

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“…Immunosuppressive therapy, such as cyclosporine, maybe useful as a first choice treatment for the treatment of CHP (10). However, the disease progression expressed by high fever, coagulopathy and cytopenia recurs often.…”

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confidence: 99%

Cytophagic Histiocytic Panniculitis -What is the Best Therapy?

Okamura

Niho

1999

Intern. Med.

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“…CHP is a life-threatening histiocytic disease of the subcutaneous adipose tissue [1] and is also thought to be one of the more severe variants of Weber-Christian disease [4]. It has been reported that many patients with CHP died as a result of massive visceral hemorrhage or organ-system failure [4,5]. CHP had been treated with glucocorticoid, including glucocorticoid pulse therapy [4].…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2011

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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.

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Successful treatment of severe cytophagic histiocyticpanniculitis with cyclosporine A

Cited by 43 publications

References 44 publications

Clonal Cytophagic Histiocytic Panniculitis in Children May Be Cured by Cyclosporine A

Clonal Cytophagic Histiocytic Panniculitis in Children May Be Cured by Cyclosporine A

Cytophagic Histiocytic Panniculitis -What is the Best Therapy?

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

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