KID综合征与化脓性汗腺炎：一种对手术治疗有反应的罕见关联
KID Syndrome and Hidradenitis Suppurativa: A Rare Association Responding to Surgical Treatment.
作者信息
Bettoli Vincenzo, Forconi Riccardo, Pezzini Ilaria, Martinello Ruby, Scuderi Valeria, Zedde Piera, Schettini Natale, Pacetti Lucrezia, Corazza Monica
机构信息
Section of Dermatology, Department of Medical Sciences, University of Ferrara, Ferrara, Italy.
Plastic and Reconstructive Surgery, Azienda Ospedaliero-Universitaria di Ferrara, Arcispedale Sant'Anna, Ferrara, Italy.
出版信息
Skin Appendage Disord. 2021 Jan;7(1):21-24. doi: 10.1159/000509042. Epub 2020 Oct 26.
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.
背景
角膜炎-鱼鳞病-耳聋（KID）综合征是一种罕见的遗传性皮肤病，其特征为角膜炎、神经性听觉障碍和鱼鳞病样皮肤受累。该综合征常见的并发症是慢性机会性皮肤感染和皮肤癌的发生。文献中描述了几例KID综合征与其他疾病的关联，包括化脓性汗腺炎（HS）。这种相关性可以用表皮的过度增殖状态来解释，这种状态在KID综合征中出现，可能有利于毛囊堵塞。
目的
本研究的目的是描述一例非常罕见的KID综合征与HS关联的病例及其复杂的治疗管理。
结果
体会到了HS常用药物治疗失败以及手术取得的良好效果，尽管手术难以实施。
结论
尽管手术存在技术难题，即皮肤的重复感染、缝线频繁裂开以及二期愈合，但作者强烈建议对这些患者采用手术治疗方法。
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