Logo American Journal of Case Reports

Call: 1.631.629.4328
Mon-Fri 10 am - 2 pm EST

Contact Us

Logo American Journal of Case Reports Logo American Journal of Case Reports Logo American Journal of Case Reports

17 August 2016 : Case report  USA

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

DOI: 10.12659/AJCR.898027

Am J Case Rep 2016; 17:587-591

Abstract

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

Add Comment 0 Comments

In Press

Case report  Belgium

Takotsubo Syndrome-Induced Cardiogenic Shock in Lung Transplantation: Importance of Early Diagnosis and ECM...

Am J Case Rep In Press; DOI: 10.12659/AJCR.944942  

Case report  China

Priapism Following Coronary Angiography: A Case Report and Clinical Insights for Diagnosis and Management

Am J Case Rep In Press; DOI: 10.12659/AJCR.943609  

Case report  USA

Anterior Mitral Line Ablation-Induced Complete Heart Block: A Cautionary Case Study

Am J Case Rep In Press; DOI: 10.12659/AJCR.945818  

Case report  South Korea

Dual-Plate Fixation in Treatment of Clavicle Fractures in Dementia Patients: A Case Series

Am J Case Rep In Press; DOI: 10.12659/AJCR.945489  

Most Viewed Current Articles

21 Jun 2024 : Case report  China (mainland) 72,630

Intracranial Parasitic Fetus in a Living Infant: A Case Study with Surgical Intervention and Prognosis Anal...

DOI :10.12659/AJCR.944371

Am J Case Rep 2024; 25:e944371

0:00

07 Mar 2024 : Case report  USA 44,111

Neurocysticercosis Presenting as Migraine in the United States

DOI :10.12659/AJCR.943133

Am J Case Rep 2024; 25:e943133

0:00

10 Jan 2022 : Case report  Germany 32,596

A Report on the First 7 Sequential Patients Treated Within the C-Reactive Protein Apheresis in COVID (CACOV...

DOI :10.12659/AJCR.935263

Am J Case Rep 2022; 23:e935263

23 Feb 2022 : Case report  USA 20,506

Penile Necrosis Associated with Local Intravenous Injection of Cocaine

DOI :10.12659/AJCR.935250

Am J Case Rep 2022; 23:e935250

Your Privacy

We use cookies to ensure the functionality of our website, to personalize content and advertising, to provide social media features, and to analyze our traffic. If you allow us to do so, we also inform our social media, advertising and analysis partners about your use of our website, You can decise for yourself which categories you you want to deny or allow. Please note that based on your settings not all functionalities of the site are available. View our privacy policy.

American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923