J Clin Rheumatol 2012 Dec;18(8):413-5
Internal Medicine and Rheumatology Unit, Nuovo Regina Margherita Hospital, Rome, Italy.
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Australas J Dermatol 2017 May 2;58(2):e54-e59. Epub 2016 Feb 2.
Clinic of Dermatovenereology, Clinical Centre of Serbia, Belgrade, Serbia.
The clinical triad of pyoderma gangrenosum, acne conglobata and hidradenitis suppurativa has been named PASH syndrome. Polycystic ovary syndrome (PCOS) is associated with hyperandrogenism and inflammation. Hidradenitis suppurativa, like acne vulgaris, may be a feature of hyperandrogenism. Read More
J Am Acad Dermatol 2012 Mar 13;66(3):409-15. Epub 2011 Jul 13.
Department of Dermatology and Allergy, Ludwig-Maximilian-University, Munich, Germany.
Background: PAPA syndrome is a recently identified hereditary autoinflammatory syndrome clinically characterized by pyogenic arthritis, severe acne, and pyoderma gangrenosum. It is caused by mutations in the PSTPIP1 gene and may be closely linked to the aseptic abscesses syndrome, which has been shown to be associated with CCTG repeat amplification in the promoter region of PSTPIP1.
Objective: We describe two unrelated patients with a clinical presentation quite similar to, yet distinct from, PAPA syndrome. Read More
Dermatol Online J 2017 Dec 15;23(12). Epub 2017 Dec 15.
Division of Dermatology, Department of Medicine, University of Stellenbosch and Tygerberg Academic Hospital, Tygerberg, Cape Town, South Africa.
PASH syndrome (pyoderma gangrenosum, acne, and suppurative hidradenitis) forms part of the spectrum of autoinflammatory diseases. We report an unusual case of PASH syndrome in a patient with end-stagerenal disease (ESRD) who was successfully treated with the tumor necrosis factor inhibitor, adalimumab. The case underscores the challenges associatedwith the treatment of PASH syndrome as well as the ongoing search to establish a genetic basis for the syndrome. Read More
Cutis 1996 May;57(5):315-9
Department of Internal Medicine, College of Medicine, University of South Florida, Tampa 33612, USA.
Pyoderma gangrenosum often presents a difficult therapeutic challenge. The case is described of a 42-year-old black man with the association of cystic acne, hidradenitis suppurativa, and seronegative arthritis with pyoderma gangrenosum. The pyoderma gangrenosum ulcers remained refractory to treatment until therapies aimed in part at the associated diseases were begun. Read More