Abstract:
UNASSIGNED: 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.
UNASSIGNED: The aim of this study was to describe a very rare case of association between KID syndrome and HS and its complex therapeutic management.
UNASSIGNED: The failure of the drugs commonly used in HS and the excellent results of surgery, although difficult to achieve, were experienced.
UNASSIGNED: 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.
摘要:
■角膜炎-鱼鳞病-耳聋(KID)综合征是一种罕见的以角膜炎为特征的遗传性皮肤病,神经感觉听觉障碍和鱼鳞病样皮肤受累。该综合征的常见并发症是慢性的,机会性皮肤感染,和皮肤癌的发展。几例KID综合征和其他疾病之间的关联,包括化脓性汗腺炎(HS),在文献中描述。这种相关性可以通过表皮的过度增殖状态来解释,发生在KID综合征中,可能有利于卵泡堵塞。
■本研究的目的是描述一个非常罕见的KID综合征与HS之间的关联及其复杂的治疗管理。
■HS中常用的药物的失败以及手术的优异效果,虽然难以实现,有经验。
■尽管与手术相关的技术困难,即,皮肤过度感染,缝合线频繁开裂,并通过次要意图关闭,作者强烈推荐这些患者的手术方法.