RESUMEN
BACKGROUND: Keratitis-ichthyosis-deafness (KID) syndrome is a rare genodermatosis characterized by keratitis, neurosensorial auditory impairment and ichthyosiform skin involvement. Frequent complications of the syndrome are chronic, opportunistic cutaneous infections, and the development of skin cancers. Several cases of association between KID syndrome and other conditions, including hidradenitis suppurativa (HS), are described in the literature. This correlation could be explained by the hyperproliferative state of the epidermis, which occurs in KID syndrome and may favor follicular plugging. OBJECTIVES: The aim of this study was to describe a very rare case of association between KID syndrome and HS and its complex therapeutic management. RESULTS: The failure of the drugs commonly used in HS and the excellent results of surgery, although difficult to achieve, were experienced. CONCLUSION: Despite the technical difficulties related to surgery, namely, cutaneous superinfections, frequent dehisce of the suture, and closure by secondary intention, the authors strongly recommend the surgical approach in these patients.