BACKGROUND: Vulvodynia, defined as vulvar pain or burning in the presence of normal vulvar appearance, is common and is associated with chronic pain syndromes and psychiatric disorders.
CASE: A postmenopausal woman complained of vulvar burning. Causes for vulvar burning including yeast infection, estrogen deficiency, and contact dermatitis were excluded. Vulvovaginal examination was normal. Subsequently, she complained of headaches, insomnia, and depression. She developed ataxic gait with rapidly progressive dementia. Brain biopsy confirmed the diagnosis of Creutzfeldt-Jakob disease, and 3 weeks later she lapsed into coma and died.
CONCLUSION: This report is unique in that a rare disease, known to result in neuronal damage, mimicked symptoms of vulvodynia in its initial phase. This supports the hypothesis that vulvodynia is a neuropathic syndrome originating in the nervous system.