关键词: "acne inversa" "hidradenitis suppurativa" PAPA PAPASH PASH PASS SAPHO arthritis autoinflammatory hidradenitis pustulosis secukinumab syndrome

Mesh : Antibodies, Monoclonal, Humanized Hidradenitis Suppurativa / drug therapy Humans Interleukin-17 Phenotype Syndrome Tumor Necrosis Factor Inhibitors

来  源:   DOI:10.12688/f1000research.52100.1   PDF(Pubmed)

Abstract:
Syndromic hidradenitis suppurativa (HS) is a form of symptom constellations, which differs from the familial and genetic form and comprises predominantly osteoarticular manifestations. Many forms include pyoderma gangrenosum and acne (PASH), pyogenic arthritis (PAPASH), spondyloarthritis (PASS) and psoriatic arthritis (PsAPASH) and are categorized in the autoinflammatory syndromes. Anti-TNF-α and anti-IL-1a blockade are between the therapeutic approaches that improve skin symptoms and prevent permanent osteoarticular damage. This case report refers to the successful treatment of a mixed phenotype of the aforementioned symptoms using the IL-17A inhibitor secukinumab after initial treatment with adalimumab. The therapy improved both cutaneous and reported osteoarticular symptoms. Different approaches for these recalcitrant HS syndromes are essential in order to achieve long-term remission for those patients.
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