Trigeminal trophic syndrome: an updated review.

Trigeminal trophic syndrome (TTS) is a rare disease process that is thought to occur after insult to the trigeminal nerve. The earliest descriptions of this condition were provided in the early 20th century, yet it remains relatively unknown, with approximately 200 cases since described. Most commonly seen in older women, TTS characteristically involves persistent facial ulceration with loss of sensation and paresthesia along the distribution of the trigeminal dermatome. Ulceration often occurs in the alar region, following self-manipulation in response to paresthesias. Time of onset of TTS after trigeminal insult may vary from weeks to decades, and emergence of ulceration may be associated with psychiatric disorders. Diagnosis is clinical and made by exclusion of similarly presenting conditions. Histology is nonspecific yet necessary to exclude other causes of facial ulceration. Although there is not yet a standard management strategy, a number of successful approaches have been reported including pharmaceutical and surgical interventions, installation of a protector, and transcutaneous nerve stimulation. However, because of the self-inflicted manifestations of this disorder, behavioral modifications remain of the utmost importance. This review serves to address the history, epidemiology, pathogenesis, clinical presentation, histology, diagnosis, differential diagnosis, and management options for TTS.