PSTPIP1-associated Arthritis, pyoderma gangrenosum and acne (PAPA), aka Pyogenic Sterile Arthritis, Pyoderma Gangrenosum, and Acne Syndrome

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SAID group:
Pyogenic Diseases


Autosomal dominant. Spontaneous mutations, with some familial groups. [29] [30]

Currently, the only documented cases are from Europe, New Zealand and the USA. [30]

Unknown, but rare.

Timing of symptoms:
Early-onset, destructive, recurrent inflammation of the joints, skin and muscle. Flares often occur after mild injury, or injections. [29]

Age of onset:
First symptoms of arthritis develop by 1-10 years of age, and skin lesions develop during adolescence. [29] [32]

Skin cutaneous:
Pathergy. Pyoderma gangrenosum ulcerative lesions, and/or severe cystic acne. Affected tissues with high neutrophil infiltration. [29]

Fevers can accompany flares of joint inflammation and pain. Other neurological symptoms are not noted. [31]

Not noted. [29] [30] [31]

Not noted. [29] [30] [31]

Not noted. [29] [30] [31]

Some patients also have irritable bowel syndrome. [29]

Not noted. [29] [30] [31]

Joints bones muscles cartilage:
Episodic inflammatory arthritis, often affecting one joint at a time that doesn’t resolve on it’s own. Intermittent sterile pauciarticular, peripheral erosive arthritis. Joint damage and destruction can often develop from the arthritis. [29] [30] [31] [32] [55]

Not noted. [29]

Not noted. [29]

Abnormal labs:
Cultures of the bone and skin are negative. Purulent synovial fluid is full of neutrophils. High with flares: CRP, ESR, WBC. [29] [30] [32]

Search Keywords:
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Photo credit:

Pyoderma Gangrenosum, often seen in PAPA - DermAtlas - Dermatology Image Atlas uploaded 9/18/2002.


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