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Granulomatosis with polyangiitis with pyoderma gangrenosum, acne, hidradenitis suppurativa-like features.
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Pyogenic arthritis, acne, pyoderma gangrenosum, and suppurative hidradenitis (PAPASH); pyogenic arthritis, pyoderma gangrenosum (PG), and acne; PG, acne, hidradenitis suppurativa; and PG, acne, spondylarthritis (PASS) are all part of a spectrum of autoinflammatory disorders that share similar pathogenesis. They are related to various mutations in the proline-serine-threonine phosphatase interacting protein 1, leading to dysregulation of the innate immune system and overproduction of interleukin (IL)-1, IL-17, and IL-23 and tumor necrosis factor (TNF)-α. Targeting these cytokines with biologics plays an important role in treatment.
View Article and Find Full Text PDFRenal involvement as a potential feature of pyogenic arthritis, pyoderma gangrenosum, and acne syndrome with E250K mutation of PSTPIP1 gene.
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Department of Dermatology, Xiangya Hospital, Central South University, Changsha, China.
Pyoderma gangrenosum, acne, and hidradenitis suppurativa syndrome is a rare inflammatory disease characterized by pyoderma gangrenosum (PG), mild to severe facial acne, and hidradenitis suppurativa (HS). It only affects the skin and represents cutaneous characteristics of a spectrum of autoinflammation. Lack of pyogenic sterile arthritis (PA) distinguishes the pyoderma gangrenosum, acne, and hidradenitis suppurativa (PASH) syndrome from pyogenic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa (PA-PASH), pyoderma gangrenosum, acne, hidradenitis suppurtiva, and ankylosing spondylitis (PASS), and pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndromes.
View Article and Find Full Text PDFCase Report: PsAPSASH syndrome: an alternative phenotype of syndromic hidradenitis suppurativa treated with the IL-17A inhibitor secukinumab.
F1000Res
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Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, Brandenburg Medical School Theodor Fontane and Faculty of Health Sciences Brandenburg, Dessau, 06847, Germany.
Article Synopsis
- - Syndromic hidradenitis suppurativa (HS) is a distinct condition characterized by a combination of symptoms, primarily affecting the bones and joints, and differs from genetic forms of HS.
- - Common forms include PASH (pyoderma gangrenosum and acne), PAPASH (pyogenic arthritis), PASS (spondyloarthritis), and PsAPASH (psoriatic arthritis), all classified under autoinflammatory syndromes.
- - This case report highlights the effective use of the IL-17A inhibitor secukinumab for treating a patient with mixed HS symptoms after initial treatment with adalimumab, resulting in improvements in skin and joint symptoms, showcasing the need for diverse treatment strategies. *
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