Recognizing Syndromic Hidradenitis Suppurativa: a review of the literature

Abstract

Background

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 etiological factors in HS.

Methods

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.

Results

We summarize 134 cases collected from the 82 included articles. The syndromes are discussed, focusing on etiopathogenesis, clinical presentation, and treatment.

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