Böck Klaus, Pschaid Carmen, Topakian Raffi, Stieglbauer Karl, Doppler Stefan, von Oertzen Joachim Tim, Pichler Robert
Department of Neurology, Wagner Jauregg Hospital, Wagner Jauregg Weg 15, 4021, Linz, Austria,
Wien Klin Wochenschr. 2014 Apr;126 Suppl 1:S42-5. doi: 10.1007/s00508-014-0504-x. Epub 2014 Mar 25.
Mononeuritis multiplex is characterized by an asymmetric pattern with affection of the peripheral nervous system; this form of polyneuropathy is often seen in non-systemic vasculitis. We present a case of multiplex neuropathy in a patient with histologicaly verified Hailey-Hailey disease. With the exception of this comorbidity--in its characteristic form presenting additionally with a superinfected subdermal node--we did not find any other possible etiologic factor possibly causative of multiplex neuritis. The diagnosis was confirmed by electrophysiological testing. To our knowledge, this is the first case report indicating a possible relationship between Hailey-Hailey disease and multiplex neuritis. There exists only one related study in the literature, which was conducted in Columbia--our patient's home country. This study delineates a clinically similar dermal disease (pemphigus foliaceus) in patients from rural Colombia (El Bagre). The authors detected anti-neuronal antibodies which were interpreted to be responsible for the pathognomonic burning sensations.
多发性单神经炎的特征是外周神经系统受累的不对称模式；这种形式的多发性神经病常见于非系统性血管炎。我们报告一例经组织学证实为黑利-黑利病患者的多发性神经病病例。除了这种合并症——其典型表现还包括一个皮下结节合并感染——我们未发现任何其他可能导致多发性神经炎的病因。电生理检查证实了诊断。据我们所知，这是首例表明黑利-黑利病与多发性神经炎之间可能存在关联的病例报告。文献中仅有一项相关研究，该研究是在我们患者的祖国哥伦比亚进行的。这项研究描述了哥伦比亚农村地区（埃尔巴格雷）患者中一种临床类似的皮肤病（落叶型天疱疮）。作者检测到抗神经元抗体，这些抗体被认为是导致特征性烧灼感的原因。
