17 August 2016 : Case report
Pyogenic Arthritis, Pyoderma Gangrenosum, Acne, Suppurative Hidradenitis (PA-PASH) Syndrome: An Atypical Presentation of a Rare Syndrome
Unusual clinical course, Challenging differential diagnosis, Rare disease, Educational Purpose (only if useful for a systematic review or synthesis)Mohammad A. UrsaniEF, Joan AppleyardE, Onome WhiteruE
Am J Case Rep 2016; 17:587-591
BACKGROUND: Pyogenic arthritis, pyoderma gangrenosum (PG), acne, and suppurative hidradenitis (PA-PASH) syndrome has been linked to an auto-inflammatory pathway. We report a case that is an atypical presentation of a rare syndrome, which supports literature suggesting that different phenotypes of PG-related syndromes may be a variation of the same pathogenic spectrum. Interestingly, our patient displayed a positive proteinase-3 antibody (PR-3). The clinical relevance of this is unclear. In recent literature, antineutrophil cytoplasmic autoantibodies (ANCA) positivity has been reported in various inflammatory conditions other than ANCA-associated vasculitis (AAV).
CASE REPORT: A 44-year-old African American male with history of pyogenic arthritis, acne, suppurative hidradenitis, and chronic diarrhea presented for evaluation of painful ulcers located on the bilateral lower extremities, bilateral proximal interphalangeal joints, buttocks, and scrotum, and chronic diarrhea. Infectious etiologies for the ulcers were ruled out. Biopsy of an ulcer revealed PG. Colonoscopy revealed inflammation and ulceration with biopsy consistent with ulcerative colitis (UC). After treatment with prednisone, the ulcers healed within 4 weeks, and the chronic diarrhea resolved.
CONCLUSIONS: Our patient displayed a variation of PA-PASH syndrome and UC. Previously reported cases of similar phenotypes of PG-related syndromes have not presented in this fashion. Furthermore, the literature does not report cases of PG-related syndromes with an elevation in PR-3 antibody. Elevation in PR-3 has been reported in various inflammatory disorders aside from AAV. The relevance of this is currently unclear. It may be possible that the milieus of these various auto-inflammatory disorders may share pathogenic commonalities.
Keywords: Arthritis, Infectious - diagnosis, Acne Vulgaris - diagnosis, Hidradenitis Suppurativa - diagnosis, Pyoderma Gangrenosum - diagnosis, Syndrome
31 January 2023 : Case reportAccelerated Cardiac Allograft Vasculopathy in an Orthotopic Heart Transplant Recipient with Prior COVID-19
Am J Case Rep In Press; DOI: 10.12659/AJCR.937955
18 January 2023 : Case reportHerpes Zoster Following COVID-19 Vaccine Booster
Am J Case Rep 2023; 24:e938667
30 December 2022 : Case reportEncephalopathy as the Only Manifestation in Simultaneous Arterial Infarct and Cerebral Venous Sinus Thrombo...
Am J Case Rep 2022; 23:e938571
03 Feb 2023 : Case reportExtraction of a Bi-Rooted Primary Maxillary Canine Tooth in a 9-Year-Old Saudi Boy: A Case Report
Am J Case Rep In Press; DOI: 10.12659/AJCR.939175
03 Feb 2023 : Case reportA 68-Year-Old Man with a Cytogenetic Diagnosis of Chronic Myeloid Leukemia and Bone Marrow Findings of Phil...
Am J Case Rep In Press; DOI: 10.12659/AJCR.938488
02 Feb 2023 : Case reportJuxtacortical White Matter Hypointensity on T2*Gradient Echo Image in Vanishing White Matter Disease: A Cas...
Am J Case Rep In Press; DOI: 10.12659/AJCR.938569
Most Viewed Current Articles
13 Jul 2022 : Case reportWhistling Scrotum: An Unusual Presentation of Pneumomediastinum in the Setting of an Open Scrotal Wound
Am J Case Rep 2022; 23:e936441
06 Dec 2021 : Case reportLipedema Can Be Treated Non-Surgically: A Report of 5 Cases
Am J Case Rep 2021; 22:e934406
23 Feb 2022 : Case reportPenile Necrosis Associated with Local Intravenous Injection of Cocaine
Am J Case Rep 2022; 23:e935250