KID Syndrome and Hidradenitis Suppurativa: A Rare Association Responding to Surgical Treatment

Abstract

<b><i>Background:</i></b> 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. <b><i>Objectives:</i></b> The aim of this study was to describe a very rare case of association between KID syndrome and HS and its complex therapeutic management. <b><i>Results:</i></b> The failure of the drugs commonly used in HS and the excellent results of surgery, although difficult to achieve, were experienced. <b><i>Conclusion:</i></b> 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.

Ocular Manifestations in Patients with Sensorineural Hearing Loss

“…[13,18] The classic dermatologic findings include vascularizing keratitis and erythrokeratoderma; however, literature has shown the presence of a follicular occlusion triad with hidradenitis suppurativa, acne conglobate, and dissecting cellulitis of the scalp. [19,20] Keratotic hyperplasic and inflammatory nodules can present on the body, which can later result in squamous cell carcinoma arising within these lesions. [21,22] Management of KID involves a multidisciplinary approach targeting ophthalmologic, otolaryngologic, and dermatologic symptoms.…”

“…Infancy, Neonatal No preference • Erythroderma [17] • Scaling and leathery skin [17] • Eryhtrodermic plaques on flexure surfaces of body [13,18] • Follicular occlusion triad [19,20] : Hidradenitis suppurativa Acne conglobate Dissecting cellulitis of the scalp • Keratotic hyperplastic and inflammatory nodules [21,22] • Squamous cell carcinoma [21,22] • Corneal vascularization 12, 16, 17, 23…”