de Golian Emily, Echols Kathryn, Pearl Henna, Davis Loretta
School of Medicine, Medical College of Georgia, Georgia Health Sciences University, Augusta, Georgia.
Pediatr Dermatol. 2014 May-Jun;31(3):373-7. doi: 10.1111/pde.12003. Epub 2012 Oct 9.
Linear atrophoderma of Moulin (LAM) is a rare dermatologic disorder characterized by a hyperpigmented atrophoderma that consistently follows the lines of Blaschko. There are many clinical and histologic similarities between LAM, atrophoderma of Pasini and Pierini (APP), and morphea, and whether LAM represents part of a disease spectrum or its own distinct entity is debated. This case of a 16-year-old boy with LAM supports the hypothesis that LAM, APP, and morphea are a spectrum of disorders rather than unique entities. Although the patient's overall clinical picture supports a diagnosis of LAM with hyperpigmented, depressed lesions following the lines of Blaschko and perivascular lymphocytic infiltrate on biopsy, the bilateral presentation typical of APP, collagen entrapment of eccrine ducts typical of morphea, and changes in dermal collagen illustrate features spanning all three disorders, suggesting a relationship between these conditions that represents a spectrum of disease. Furthermore, a review of all reported cases of LAM in the literature suggests an evolving definition beyond what Moulin and colleagues originally described, including features related to those of APP and morphea.
穆兰线性皮肤萎缩症（LAM）是一种罕见的皮肤病，其特征为色素沉着性皮肤萎缩，且始终沿布拉斯科线分布。LAM、帕西尼和皮耶里尼皮肤萎缩症（APP）以及硬斑病在临床和组织学上有许多相似之处，LAM究竟是疾病谱的一部分还是一种独立的疾病，目前仍存在争议。该例16岁患LAM的男孩支持了以下假说，即LAM、APP和硬斑病是一系列疾病而非独立的病种。尽管患者的整体临床表现支持LAM的诊断，其色素沉着、凹陷性皮损沿布拉斯科线分布，活检显示血管周围淋巴细胞浸润，但具有APP典型的双侧表现、硬斑病典型的汗腺导管胶原包埋以及真皮胶原变化，这些特征跨越了所有这三种疾病，提示这些病症之间存在一种代表疾病谱的关系。此外，对文献中所有已报道的LAM病例进行回顾发现，其定义正在演变，超出了穆兰及其同事最初所描述的范围，包括与APP和硬斑病相关的特征。
