Vollono Laura, Iorizzo Matilde, Richert Bertrand
Private Dermatology Practice, Rome, Italy.
Private Dermatology Practice, Bellinzona/Lugano, Switzerland.
Skin Appendage Disord. 2024 Oct;10(5):335-341. doi: 10.1159/000538553. Epub 2024 May 20.
There are few studies, mainly case reports, on the involvement of the nail unit in autoimmune bullous disorders.
Nail involvement in autoimmune bullous disorders is a significant clinical phenomenon, marked by a range of manifestations, most often not presenting with blisters like on the skin but rather with alterations of the nail unit such as paronychia, onychomadesis, or onycholysis. This involvement is particularly notable due to the unique immunological features of the nail unit, including the expression of various antigens and the presence of Langerhans cells. Conditions like pemphigus vulgaris, bullous pemphigoid, epidermolysis bullosa acquisita, linear IgA disease, and bullous systemic lupus erythematosus can lead to nail abnormalities. The prevalence of nail manifestations varies according to the disorder, and diagnosis often relies on histopathological and immunofluorescence testing. Nail involvement correlates with disease severity and duration, sometimes serving as a herald sign. Further research is needed to guide therapeutic approaches for nail involvement in autoimmune bullous diseases.
Nail involvement in autoimmune bullous nail disorders may be confusing as there are almost never bullae. One should keep the diagnosis in mind when facing an atypical paronychia.
关于甲单位在自身免疫性大疱性疾病中的受累情况，研究较少，主要是病例报告。
甲在自身免疫性大疱性疾病中的受累是一种重要的临床现象，其表现多样，最常见的不是像皮肤那样出现水疱，而是甲单位出现改变，如甲沟炎、甲脱落或甲剥离。由于甲单位独特的免疫学特征，包括各种抗原的表达和朗格汉斯细胞的存在，这种受累情况尤为显著。寻常型天疱疮、大疱性类天疱疮、获得性大疱性表皮松解症、线状IgA病和大疱性系统性红斑狼疮等疾病可导致甲异常。甲表现的患病率因疾病而异，诊断通常依赖于组织病理学和免疫荧光检测。甲受累与疾病严重程度和病程相关，有时可作为先兆体征。需要进一步研究以指导自身免疫性大疱性疾病中甲受累的治疗方法。
甲在自身免疫性大疱性甲病中的受累情况可能令人困惑，因为几乎从不出现大疱。面对非典型甲沟炎时应考虑到这一诊断。
