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J Eur Acad Dermatol Venereol. 2015 Nov;29(11):2243-7. doi: 10.1111/jdv.12765. Epub 2014 Oct 28.

Successful treatment of PASH syndrome with infliximab, cyclosporine and dapsone.

Author information

1
Department of Dermatology, University Medical Center Mannheim, Medical Faculty Mannheim, University of Heidelberg, Germany.
2
Department of Dermatology and Venereology, Federal Academic Teaching Hospital, Feldkirch, Austria.
3
Department of Dermatology and Allergy, Ludwig-Maximilian University, Munich, Germany.
4
Institute for Laboratory Medicine and Human Genetics, Singen, Germany.

Abstract

BACKGROUND:

The group of autoinflammatory syndromes associated with Pyoderma gangrenosum, Acne, and Suppurative Hidradenitis are poorly defined and difficult to control with currently available treatment modalities.

OBJECTIVES:

We describe a patient with PASH syndrome and report about the successful multimodal treatment with infliximab, cyclosporine, and dapsone.

METHODS:

A review of the available literature to date about this group of autoinflammatory diseases was performed. We performed genetic analysis for PSTPIP1 mutations associated with PAPA syndrome.

RESULTS:

A 22-year-old woman presented to our department with pyoderma gangrenosum, concomitant acne, and suppurative hidradenitis. She had previously been treated unsuccessfully with etanercept, adalimumab, fumaric acid and the IL-1 receptor antagonist (IL-1RA) anakinra without prolonged remission. Treatment with intravenous infliximab in combination with cyclosporine and dapsone lead to sudden and prolonged improvement of the clinical symptoms that we classified as PASH syndrome. We review the literature about this group of diseases and report the third case of PASH syndrome to date.

CONCLUSION:

PASH syndrome and associated diseases should be considered whenever hidradenitis suppurativa is found in association with pyoderma gangrenosum. We provide a systematic overview about PASH syndrome and suggest a novel multimodal therapeutic regimen beyond isolated inhibition of TNF or IL-1.

PMID:
25352307
DOI:
10.1111/jdv.12765
[Indexed for MEDLINE]

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