PAPA syndrome


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Related to PAPA syndrome: SAPHO syndrome

PAPA syndrome

(pah'pah sin'drōm),
An autosomal dominant syndrome consisting of pyogenic sterile arthritis, pyoderma gangrenosum, and acne.
A rare autosomal dominant [MIM 64416] condition characterised by the acronym PAPA:
pyogenic arthritis
pyoderma gangrenosum and
cystic acne
Lab Hypogammaglobulinaemia, elevated tumour necrosis factor-alpha
Management Intra-articular corticosteroid therapy, methotrexate, colchicine
References in periodicals archive ?
PAPA syndrome not only affects Lily's joints but her immune system is severely affected too, so the slightest infection could have serious consequences.
Mansfield et al., "Pyrin binds the PSTPIP1/CD2BP1 protein, defining familial Mediterranean fever and PAPA syndrome as disorders in the same pathway," Proceedings of the National Academy of Sciences of the United States of America, vol.
Seidman et al., "Mutations in CD2BP1 disrupt binding to PTP PEST and are responsible for PAPA syndrome, an autoinflammatory disorder," Human Molecular Genetics, vol.
Pascual, "Dramatic improvement of pyoderma gangrenosum with infliximab in a patient with PAPA syndrome," Pediatric Dermatology, vol.
Pyoderma gangrenosum, acne, and suppurative hidradenitis (PASH)-a new autoinflammatory syndrome distinct from PAPA syndrome. J Am Acad Dermatol 2012;66:409-15.
McEvov, "A new autosomal dominant disorder of pyogenic sterile arthritis, pyoderma gangrenosum, and acne: PAPA syndrome," Mayo Clinic Proceedings, vol.
Kuhns et al., "Brief report: genotype, phenotype, and clinical course in five patients with PAPA syndrome (pyogenic sterile arthritis, pyoderma gangrenosum, and acne)," Arthritis & Rheumatism, vol.