Recognizing syndromic hidradenitis suppurativa: a review of the literature

Abstract

Hidradenitis suppurativa (HS) is an inflammatory skin disease causing painful inflammation and suppuration. It may occur in rare syndromes: follicular occlusion, Bazex-Dupré-Christol, Down's, KID, PAPASH, PASS, PASH, and SAPHO syndromes, as well as Dowling-Degos disease. An overview of syndromic HS may inform the search for aetiological factors in HS. PubMed, Ovid and Web of Science were systematically searched using '(hidradenitis OR acne invers*) AND (syndrome OR KID OR PASS OR PAPA OR PASH OR SAPHO OR bazex-dupre OR 'dowling degos' OR triad OR tetrad)' and Cochrane Library using 'hidradenitis OR acne invers*'. A total of 82 articles were included in the final review. We summarize 134 cases collected from the 82 included articles. The syndromes are discussed, focusing on etiopathogenesis, clinical presentation and treatment. This study is based on case reports; therefore, conclusions may be subject to the selection bias. These syndromes are rare; however, it is important to recognize them, as treating them may require a different approach. Three subtypes of syndromic hidradenitis are suggested: syndromes with known genetic abnormalities, syndromes characterized by follicular plugging or structural defects, and syndromes with possible autoinflammatory pathogenesis. There was no universally effective treatment for syndromic HS, and treatment was individualized.