Pyogenic Arthritis, Pyoderma Gangrenosum, and Acne (PAPA) Syndrome

2020 ◽  
pp. 564-568
Author(s):  
Deborah L. Stone ◽  
Daniel L. Kastner
Keyword(s):  
Pyoderma Gangrenosum ◽  
Papa Syndrome ◽  
Pyogenic Arthritis

Republished: Cerebral vascular findings in PAPA syndrome: cerebral arterial vasculopathy or vasculitis and a posterior cerebral artery dissecting aneurysm

2015 ◽  
Vol 8 (8) ◽  
pp. e29-e29 ◽  
Author(s):  
Kasra Khatibi ◽  
Jeremy J Heit ◽  
Nicholas A Telischak ◽  
Jorina M Elbers ◽  
Huy M Do
Keyword(s):  
Subarachnoid Hemorrhage ◽  
Young Patient ◽  
Pyoderma Gangrenosum ◽  
Cerebral Artery ◽  
Posterior Cerebral Artery ◽  
Dissecting Aneurysm ◽  
Neurologic Deficit ◽  
Papa Syndrome ◽  
Pyogenic Arthritis ◽  
Cerebral Vascular

A young patient with PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne) syndrome developed an unusual cerebral arterial vasculopathy/vasculitis (CAV) that resulted in subarachnoid hemorrhage from a ruptured dissecting posterior cerebral artery (PCA) aneurysm. This aneurysm was successfully treated by endovascular coil sacrifice of the affected segment of the PCA. The patient made an excellent recovery with no significant residual neurologic deficit.

Imaging findings of sterile pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome: differential diagnosis and review of the literature

2018 ◽  
Vol 49 (1) ◽  
pp. 23-36 ◽  
Author(s):  
Claudia Martinez-Rios ◽  
Mehul P. Jariwala ◽  
Kerri Highmore ◽  
Karen Watanabe Duffy ◽  
Lynn Spiegel ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Pyoderma Gangrenosum ◽  
Imaging Findings ◽  
Review Of The Literature ◽  
Papa Syndrome ◽  
Pyogenic Arthritis

scholarly journals A case of pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome successfully treated with combination therapy of corticosteroids, cyclosporine, and colchicine

2018 ◽  
Vol 2 (2) ◽  
Author(s):  
Hideo Hashizume ◽  
Reiko Kageyama ◽  
Takatsune Umayahara ◽  
Tomohiro Morio
Keyword(s):  
Combination Therapy ◽  
Clinical Features ◽  
Pyoderma Gangrenosum ◽  
Therapeutic Option ◽  
Combined Therapy ◽  
Genetic Mutation ◽  
Papa Syndrome ◽  
Destructive Inflammation ◽  
Factor Α ◽  
Pyogenic Arthritis

Pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome is an autoinflammatory disease characterized by destructive inflammation of the skin and joints in association with genetic mutation of the Pombe Cdc15 homology family member PSTPIP1. Because a therapeutic strategy specific to this disease has not been established, treatment is always challenging for clinicians. We herein describe a case of PAPA syndrome with typical clinical features successfully treated with combination therapy of traditional anti-inflammatory drugs. A 39-year-old man presented with painful plaques on his extremities that had been present for several years. Large brown plaques were observed on both arms and legs with numerous fistulae and ulcers. Cystic acne lesions subsequently appeared on his cheeks and upper back. We diagnosed the patient with PAPA syndrome based on the presence of typical clinical features; however, no genetic mutations of exon-1 to 15 of PSTPIP1were found. Although recent reports have emphasized the efficacy of biologics that target inflammatory cytokines such as antibodies to interleukin-1β and tumor necrosis factor-α, use of these agents remains uncovered by health insurance in Japan, showing unresolved discrepancy in practical use for clinicians. The present patient was successfully treated with combined therapy of a corticosteroid, colchicine, and cyclosporine A, encouraging the use of this combination therapy as a novel therapeutic option.

scholarly journals Case Report: Pyogenic Arthritis, Pyoderma Gangrenosum, and Acne: A Single-Center Experience and Literature Review

2021 ◽  
Vol 12 ◽  
Author(s):  
Yumei Wang ◽  
Na Wu ◽  
Keyi Yu ◽  
Min Shen
Keyword(s):  
Literature Review ◽  
Pyoderma Gangrenosum ◽  
Skin Lesions ◽  
Single Center ◽  
Intermittent Fever ◽  
Chinese Han ◽  
Full Spectrum ◽  
Papa Syndrome ◽  
Classical Triad ◽  
Pyogenic Arthritis

ObjectivesThis study aims to describe the characteristics of patients diagnosed with pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome at a single center in China and provide an up-to-date literature review.MethodsThe clinical data and genotype of three Chinese Han patients were carefully documented and studied. We also conducted a systematic literature review on PAPA syndrome.ResultsA total of three patients were diagnosed with PAPA syndrome at our center from 2018 to 2020. Arthritis was observed in all three patients, while pyoderma gangrenosum (PG) was found in two patients and acne in one patient. Other manifestations included pathergy reaction, intermittent fever, oral ulcer, keratitis, proteinuria, and hematuria. The PSTPIP1 A230T mutation was identified in two patients, and a novel Y119C variation was revealed in a sporadic patient. A total of 76 patients with PAPA syndrome reported in 29 articles were included in our literature review. The classical triad of arthritis, PG, and acne was visible in only 16 (25.4%) patients, while 24 (38.1%) exhibited only one major symptom. Skin lesions were more commonly seen in patients with adult-onset disease than those with childhood-onset disease (100 vs. 83%), whereas arthritis was less common (50 vs. 98.1%). Steroid and/or biological agents were effective in most patients.ConclusionsThe rarity and phenotypic heterogeneity associated with PAPA syndrome make the diagnosis a huge challenge to physicians, especially in adult patients. A significant portion of patients did not exhibit the full spectrum of the classical triad. Accordingly, gene testing is critically helpful for diagnosis.

Novel PSTPIP1 gene mutation in a patient with pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome

2015 ◽  
Vol 45 (1) ◽  
pp. 91-93 ◽  
Author(s):  
Elvira Lindwall ◽  
Shikha Singla ◽  
William E. Davis ◽  
Robert J. Quinet
Keyword(s):  
Gene Mutation ◽  
Pyoderma Gangrenosum ◽  
Papa Syndrome ◽  
Pyogenic Arthritis
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