Asena ÜNAL, Hilal BAŞLI, Ümmühan ÇAY, Özlem Özgür GÜNDEŞLİOĞLU, Derya ALABAZ, Miray Kışla EKİNCİ, Bilge Fettahlıoğlu KARAMAN
Turkish Archives of Pediatrics - 2026;61(3):272-274
Hidradenitis Suppurativa (HS) is a chronic, recurrent, and painful inflammatory skin disease that typically presents with deep-seated nodules and abscesses, which may lead to the formation of sinus tracts and scarring. It most commonly occurs after adolescence in areas rich in apocrine sweat glands, such as the axillae, inguinal folds, and anogenital region. HS is rarely associated with autoinflammatory syndromes. However, with the increasing number of cases reporting the coexistence of HS and autoinflammatory diseases in recent years, along with a better understanding of its etiopathogenesis, inflammation resulting from the hyper-activation of innate immunity due to genetic factors can occasionally lead to inflammatory keratinization disorders of the skin, referred to as "autoinflammatory keratinization diseases" (AiKD). HS is now considered a subtype of AiKD. The mevalonate kinase gene (MVK), which is thought to contribute to the development of HS, is also implicated in Hyperimmunoglobulin D syndrome (HIDS). In this paper, the case of a 15-year-old girl who had been followed for HIDS for 6 years and was subsequently diagnosed with HS during an investigation for recurrent left axillary abscesses over the past 1.5 years is presented.
