背景:板层鱼鳞病是一种罕见的先天性疾病,整形外科医生在日常实践中可能会遇到。其临床诊断使其成为识别和知道如何治疗的重要病理学。
方法:我们报告了一例患有层状鱼鳞病并伴有头皮糜烂性假性脓疱病的患者。我们的治疗方案采用两次相隔三个月的病灶内延迟皮质激素(Kenacort®)注射,显示出病变的临床明显改善。
结论:先天性板层鱼鳞病将各种临床表现重新分组。文献中描述的大多数治疗策略涉及局部和全身治疗。权衡患者,并导致适度的结果。也已经报道了手术治疗或透明质酸注射,但是它们引起了关于发病率和效率的问题。
结论:我们通过两次Kenacort®注射三个月间隔的治疗策略很简单,可再现,并在治疗患有先天性层状鱼鳞病并伴有头皮糜烂性假性脓疱病的患者中显示出效率。
BACKGROUND: Lamellar ichthyosis is a rare congenital disorder that can be encountered by plastic surgeon in a daily practice. Its clinical diagnosis makes it an significant pathology to identify and to know how to treat.
METHODS: We report the case of a patient suffering from lamellar ichthyosis complicated by erosive pseudo pustulosis of the scalp. Our treatment protocol with two intra-lesional delayed-corticoids (Kenacort ®) injections three months apart showed significant clinical improvement of the lesions.
CONCLUSIONS: Congenital lamellar ichthyosis regroups various clinical presentations. Most of the therapeutic strategies described in the literature involve local and systemic treatments, weighing on patients and leading to modest results. Surgical treatment or hyaluronic injections have also been reported but they raise problematics regarding morbidity and efficiency.
CONCLUSIONS: Our therapeutic strategy by two Kenacort ® injections three months apart is simple, reproductible and has shown efficiency in the treatment of our patient suffering from congenital lamellar ichthyosis complicated with erosive pseudo pustulosis of the scalp.