硬化萎缩性苔藓并发局限性硬皮病1例并文献复习

Lichen sclerosus et atrophicus co-existing with localized scleroderma:a case report and literature review

目的探讨硬化萎缩性苔藓(LSA)并发局限性硬皮病(LoS)的临床表现、组织病理学特点及诊断,提高对本病的认识,更好地理解硬皮病的疾病谱。方法分析1例LSA并发LoS的临床表现及组织病理特点,复习相关文献并进行讨论。结果患者全身皮损进行性增多无痛痒5年余。2年前初次就诊时明确诊断为LSA,治疗后皮损仍进行性增多,且硬化。此次就诊时皮肤科检查:躯干、四肢散在或密集粟粒至黄豆大瓷白色、略带光泽的扁平丘疹,部分萎缩、硬化;四肢、左手足片状或带状暗红色硬化性斑,部分皮损上有瓷白色扁平丘疹。初次就诊时背部皮损组织病理检查结果显示符合LSA。此次就诊时左小腿皮损组织病理检查结果符合LSA并发硬皮病。结合临床表现和组织病理检查结果,诊断为LSA并发LoS。结论该例LSA并发LoS的患者临床过程、临床表现和组织病理学改变表明,硬皮病可能是谱系性疾病,或许可以认为LSA是浅表性硬皮病。

Objective To investigate the clinical manifestations,pathological features and diagnosis of lichen sclerosus et atrophicus（LSA） co-existing with localized scleroderma（Lo S）,so as to raise the awareness of the disease and better understand the spectrum of scleroderma.Methods A case of LSA co-existing with Lo S was reported.The clinical data and histopathological features of the case were analyzed.The relevant literatures were reviewed while the discussions were conducted.Results The patient was introduced to our department because of a more than 5-year history of progressive increasing skin lesions over the whole body which without pain or itch.Two years ago,the patient was first diagnosed as LSA in our department.The skin lesions responded poorly to treatment,which progressively increased and became harden.In the last visit,physical examination showed separate or dense,from millet to soybean in diameter,porcelain white,slightly sheen and flat papules which partially atrophied and harden over the trunk and extremities; sheet or band-like,dark red and sclerous patches on which porcelain white and flat papules could be seen over the extremities,left hand and foot.In the first visit,the histopathology of the skin lesions on the back showed correspondence to diagnosis with LSA.In the last visit,the histopathology of the skin lesions on the left calf showed correspondence to diagnosis with LSA co-existing with scleroderma.According to the clinical data and histopathological features,the patient was diagnosed as LSA co-existing with Lo S.Conclusion The clinical processes,clinical manifestations and pathological features of the case more clearly indicated that scleroderma might be a spectrum of disorder and perhaps LSA could be considered as superficial scleroderma.