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01.04.2014 | case report | Sonderheft 1/2014

Wiener klinische Wochenschrift 1/2014

Mononeuritis multiplex: association with infectious condition and familial background in a tropical environment: a case report

Wiener klinische Wochenschrift > Sonderheft 1/2014
MD Klaus Böck, MD Carmen Pschaid, MD Raffi Topakian, MD Karl Stieglbauer, Stefan Doppler, MD, FRCP Joachim Tim von Oertzen, MD, PhD Robert Pichler


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.

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