Böhm M
Klinik für Hautkrankheiten, Universitätsklinikum Münster, Von-Esmarch-Str. 58, 48149, Münster, Deutschland.
Hautarzt. 2015 Dec;66(12):945-58; quiz 959-60. doi: 10.1007/s00105-015-3717-7.
Hypomelanoses of the skin encompass a wide spectrum of congenital and acquired alterations in melanin pigmentation. These diseases can be localized or universal. The pathobiology of cutaneous hypomelanoses is heterogeneous and includes defects in melanoblast migration from the neural crest to the epidermis, alterations in melanogenesis and melanin transfer to keratinocytes, and destruction of pigment cells by autoimmune and inflammatory processes. Importantly, some congenital forms of universal hypomelanoses are associated with involvement of internal organs (e.g., Hermansky-Pudlack or Chédiak-Higashi syndrome) and require interdisciplinary patient management. In recent years, significant progress has been made in our current understanding of the pathophysiology especially of vitiligo, thus, resulting in promising new treatment strategies. This disease being one of the most common forms of acquired hypomelanoses of the skin can now be treated in a guideline-oriented and evidence-based manner.
皮肤色素减退症涵盖了黑色素沉着方面多种先天性和后天性改变。这些疾病可为局限性或全身性的。皮肤色素减退症的病理生物学具有异质性,包括黑素母细胞从神经嵴迁移至表皮过程中的缺陷、黑素生成及黑色素向角质形成细胞转移的改变,以及自身免疫和炎症过程导致的色素细胞破坏。重要的是,一些全身性色素减退症的先天性形式与内脏受累有关(如Hermansky-Pudlack综合征或Chédiak-Higashi综合征),需要多学科的患者管理。近年来,我们对病理生理学的认识,尤其是对白癜风的认识取得了重大进展,从而产生了有前景的新治疗策略。这种疾病作为皮肤后天性色素减退症最常见的形式之一,现在可以按照以指南为导向且基于证据的方式进行治疗。