Lipsker D, Piette J C, Laporte J L, Maunoury L, Francès C
Service de Médecine Interne, Hôpital Pitié-Salpëtrière, Paris, France.
Dermatology. 1997;195(4):402-3. doi: 10.1159/000245999.
We report the case of a patient who had 2 rare diseases, annular atrophic lichen planus (AALP) and Sneddon's syndrome (SNS). This patient had also digital nodules with histological abnormalities suggestive of SNS vasculopathy, which have not been reported so far. AALP is the most rare of all varieties of lichen planus since this case is the third reported to date. The association of livedo racemosa and cerebrovascular disease is the hallmark of SNS, the incidence of which is estimated to be 4 cases per year per million inhabitants. In both diseases, an abnormal production of elastic-tissue-degrading enzymes or a constitutional abnormality of the elastic tissue can be postulated, since SNS is characterized by arteriolar changes with deterioration of the internal elastic lamina and AALP by destruction of the dermal elastic tissue.
我们报告了一例患有两种罕见疾病的患者，即环状萎缩性扁平苔藓（AALP）和斯内登综合征（SNS）。该患者还出现了具有组织学异常的指端结节，提示SNS血管病变，迄今为止尚未见相关报道。AALP是所有扁平苔藓类型中最为罕见的，因为该病例是迄今为止报道的第三例。网状青斑与脑血管疾病的关联是SNS的标志，其发病率估计为每百万居民每年4例。在这两种疾病中，可以推测存在弹性组织降解酶的异常产生或弹性组织的体质性异常，因为SNS的特征是小动脉改变伴内弹性膜退化，而AALP的特征是真皮弹性组织破坏。
