Mismedicine Organization and Research Institute, Beverly Hills, CA, USA.
Adv Exp Med Biol. 2022;1367:155-172. doi: 10.1007/978-3-030-92616-8_7.
Morphea and lichen sclerosis et atrophicus (LSA) are two distinct immune-mediated diseases with a dominant presentation of dermal fibrosis and sclerosis. The two diseases have many similar clinical and histological features and tend to co-occur. Both diseases are thought to result from a derailment of the normal response to environmental triggers. Positive family history is more common in LSA than morphea but individuals with morphea have a higher frequency of concomitant and familial autoimmunity. These findings hint at the involvement of inheritance in susceptibility to LSA and morphea and thus provide a rationale for exploring the disease genetics. This chapter contains a comprehensive review of the pathogenesis of the two diseases and their known genetic associations including HLA class I and II genes.
硬斑病和线状硬斑病（LSA）是两种不同的免疫介导性疾病，以皮肤纤维化和硬化为主要表现。这两种疾病具有许多相似的临床和组织学特征，并且往往同时发生。这两种疾病都被认为是对环境触发因素的正常反应失调所致。线状硬斑病的家族史阳性率高于硬斑病，但硬斑病患者自身免疫的伴发率和家族聚集率更高。这些发现提示遗传因素可能与线状硬斑病和硬斑病的易感性有关，从而为探索疾病遗传学提供了依据。本章全面回顾了这两种疾病的发病机制及其已知的遗传关联，包括 HLA Ⅰ类和Ⅱ类基因。
