Pemphigus is associated with the development of certain neurologic diseases, in particular, dementia, epilepsy, and Parkinson disease (PD), according to the results of a retrospective, population-based, cross-sectional study published in JAMA Dermatology.

The investigators evaluated the association between pemphigus and 4 neurologic diseases: dementia, epilepsy, PD, and multiple sclerosis (MS), examining one of the largest cohorts of patients with pemphigus.

The analysis was performed between January 1, 2004, and December 31, 2014, using information from the Clalit Health Services of Israel. A total of 1985 patients newly diagnosed with pemphigus and 9874 controls were enrolled in the study. 

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The number of participants with dementia, epilepsy, PD, and MS were compared in those diagnosed with pemphigus vs age-, gender-, and ethnicity-matched controls.

When patients with pemphigus (1188 women and 797 men; mean age, 72.1±18.5 years) were compared with controls (5912 women and 3962 men; mean age, 72.1±18.5 years), there was a 2-fold increase in the odds of the following neurologic conditions developing: dementia (31.3% [622 of 1985] of those with pemphigus vs 18.8% [1856 of 9874] of controls; objective response (OR) 1.97; 95% CI, 1.77-2.20); epilepsy (3.7% [74 of 1985] of those with pemphigus vs 2.1% [210 of 9874] of controls; OR 1.78; 95% CI, 1.36-2.33); and PD (8.8% [175 of 1985] of those with pemphigus vs 4.4% [437 of 9874] of controls; OR 2.09; 95% CI, 1.74-2.51).

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In contrast, MS was reported in 0.1% (2 of 1985) of those with pemphigus vs 0.01% (6 of 9874) of controls (OR 1.65; 95% CI, 0.34-8.22).

The investigators concluded that clinicians should be aware of the association between pemphigus and these 3 neurologic disorders. Additional observational research is warranted to better understand the molecular mechanism behind this link.


Kridin K, Zelber-Sagi S, Comaneshter D, Cohen AD. Association between pemphigus and neurologic diseases [published online February 16, 2018]. JAMA Dermatol. doi: 10.1001/jamadermatol.2017.5799