Severe pemphigoid gestationis associated with acute disseminated encephalomyelitis in the setting of a systemic disorder.

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Pemphigoid gestationis is a skin-specific autoimmune disorder that can sometimes present as the cutaneous manifestation of a multiorgan disease due to potentially common pathogenic mechanisms. We report a severe form of pemphigoid gestationis in a 32-year-old primigravida woman, who presented at 22 weeks of gestation with headaches and blurred vision, later developing encephalitis, intrauterine fetal demise and dilated cardiomyopathy.

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