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[硬化萎缩性苔藓——一个诊断难题]

[Lichen sclerosus et atrophicus--a diagnostic problem].

作者信息

Golusin Z, Jovanović M, Vujanović J, Subotić M

机构信息

Klinika za kozno-venericne bolesti, Klinicki centar, Medicinski fakultet, Novi Sad.

出版信息

Med Pregl. 2001 Mar-Apr;54(3-4):187-90.

Abstract

INTRODUCTION

Lichen sclerosus et atrophicus (LSA) is a rare disease with etiology that has not been clearly defined up to now. This disease appears up to 10 times more frequently among women, than among men. It occurs at the age of 40-60. Anogenital site is the most common, but in 20% of cases it is extragenital.

CASE REPORT

We report a case of a 78-year-old woman with skin lesions on the hand and face, that appeared 7-8 years ago after intensive sun exposure. When admitted to hospital, she had a butterfly-shaped livid erythema on cheeks and nose hypopigmentation on its edges. Atrophic plaques were formed in some areas of lesions. On the dorsal side of hand clear atrophic plaques were noticed. Pathohistological analysis of skin lesions revealed epidermal hyperkeratosis and atrophy with follicular hyperkeratosis, dermal edema, with subepidermal blistering and edematous hyalinized connective tissue. Direct immunofluorescence of the face lesion has shown presence of IgA, IgG and IgM deposits along the epidermo-dermal interface. Based on all findings the following diagnosis was made: Lichen sclerosus et atrophicus bullosus.

DISCUSSION

Differentiation between chronic discoid lupus erythematosus and LSA presents a diagnostic challenge. Both diseases have some common pathohistological changes, but a single mixed inflammatory dermal infiltrate as well as edema and hyalinized connective tissue were the most important facts in making diagnosis.

CONCLUSION

Lack of knowledge in regard to etiology of this disease has caused some problems in differential diagnosis. This disease hasn't always been a separate entity, but we expect that many things regarding the etiology and pathogenesis to be explained in the future.

摘要

引言

硬化萎缩性苔藓(LSA)是一种罕见疾病,其病因至今尚未明确。这种疾病在女性中的发病率比男性高10倍。发病年龄在40至60岁之间。肛门生殖器部位最为常见,但20%的病例发生在生殖器外。

病例报告

我们报告一例78岁女性,手部和面部出现皮肤病变,这些病变于7至8年前在强烈日晒后出现。入院时,她脸颊上有蝶形青紫色红斑,边缘有色素减退,病变某些区域形成了萎缩性斑块。在手部背侧可见明显的萎缩性斑块。皮肤病变的病理组织学分析显示表皮角化过度和萎缩,伴有毛囊角化过度、真皮水肿、表皮下水疱形成以及水肿性玻璃样变的结缔组织。面部病变的直接免疫荧光显示在表皮-真皮界面有IgA、IgG和IgM沉积。基于所有这些发现,做出了以下诊断:大疱性硬化萎缩性苔藓。

讨论

慢性盘状红斑狼疮和LSA之间的鉴别诊断具有挑战性。这两种疾病有一些共同的病理组织学变化,但单一混合性真皮炎性浸润以及水肿和玻璃样变的结缔组织是做出诊断的最重要依据。

结论

对这种疾病病因的认识不足在鉴别诊断中造成了一些问题。这种疾病并非一直是一个独立的病种,但我们期望未来能对其病因和发病机制有更多的解释。

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