Istituto Dermopatico dell'Immacolata-IRCCS, FLMM, Rome, Italy.
J Dermatol. 2018 Sep;45(9):1135-1140. doi: 10.1111/1346-8138.14529. Epub 2018 Jul 14.
The clinical features of bullous pemphigoid are extremely polymorphous. Several atypical forms of bullous pemphigoid have been described, and the diagnosis critically relies on immunopathological findings. We describe three bullous pemphigoid patients characterized by palmoplantar keratoderma, diffused hyperkeratotic cutaneous lesions and extremely high levels of immunoglobulin E serum. The diagnosis of bullous pemphigoid should be taken into account in patients presenting diffused hyperkeratotic cutaneous lesions and palmoplantar keratoderma, even in the absence of blisters. Alteration of the keratinization process, that could occur in patients with genetic mutations in desmosomal and hemidesmosomal genes, may also be due to circulating autoantibodies against hemidesmosomal proteins in these bullous pemphigoid patients.
大疱性类天疱疮的临床特征极其多样。已经描述了几种非典型形式的大疱性类天疱疮,诊断主要依赖于免疫病理学发现。我们描述了 3 例大疱性类天疱疮患者,其特征为手掌足底角化过度、弥漫性角化过度性皮肤病变和极高水平的血清免疫球蛋白 E。在无大疱的情况下,对于出现弥漫性角化过度性皮肤病变和手掌足底角化过度的患者,应考虑大疱性类天疱疮的诊断。角化过程的改变,可能发生在桥粒和半桥粒基因中的基因突变患者中,也可能是由于这些大疱性类天疱疮患者中存在针对半桥粒蛋白的循环自身抗体。