scholarly journals Brief Report: Genotype, phenotype, and clinical course in five patients with PAPA syndrome (pyogenic sterile arthritis, pyoderma gangrenosum, and acne)

2012 ◽  
Vol 64 (6) ◽  
pp. 2022-2027 ◽  
Author(s):  
Andrew P. Demidowich ◽  
Alexandra F. Freeman ◽  
Douglas B. Kuhns ◽  
Ivona Aksentijevich ◽  
John I. Gallin ◽  
...  
Keyword(s):  
Pyoderma Gangrenosum ◽  
Clinical Course ◽  
Papa Syndrome

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.

A New Autosomal Dominant Disorder of Pyogenic Sterile Arthritis, Pyoderma Gangrenosum, and Acne: PAPA Syndrome

1997 ◽  
Vol 72 (7) ◽  
pp. 611-615 ◽  
Author(s):  
Noralane M. Lindor ◽  
Todd M. Arsenault ◽  
Herman Solomon ◽  
Christine E. Seidman ◽  
Marian T. McEvoy
Keyword(s):  
Pyoderma Gangrenosum ◽  
Autosomal Dominant ◽  
Autosomal Dominant Disorder ◽  
Papa Syndrome

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.

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