Trigeminal Trophic Syndrome—A Unique Clinical Presentation of a Rare Condition

Abstract

This study describes a unique clinical presentation of trigeminal trophic syndrome (TTS), which is not well described within the otolaryngology literature. Trigeminal trophic syndrome classically presents with a triad of symptoms: trigeminal anesthesia, facial paresthesias, and crescent-shaped ulceration of the lateral nasal ala. The patient discussed in this report had a self-induced, waxing and waning ulceration of the frontal scalp for 7 years and was evaluated and treated ineffectively by multiple physicians, including otolaryngologists, before TTS was diagnosed and a targeted treatment was initiated. Although extranasal presentation is uncommon, this condition must be considered when ulcers are encountered in the trigeminal dermatome. This case highlights the variability in presentation and the importance of awareness of this rare syndrome. We aim to facilitate more prompt diagnosis and expedite the initiation of appropriate treatment for TTS in the field of otolaryngology.

Trigeminal trophic syndrome: an important simulator of discoid cutaneous lupus erythematosus – a case series

“…TTS more commonly presents with trigeminal anaesthesia, facial paraesthesia and ulceration of the lateral nasal ala, generally sparing the tip of the nose. 2–5 TTS is often misdiagnosed as other diseases before the correct diagnosis is achieved. Patients are initially suspected of having chronic cutaneous LE, cutaneous tumours, chronic granulomatous infections or dermatitis artefacta, and are commonly referred from and for psychodermatology clinics.…”

“…The latency period between the trigeminal nerve lesion and the onset of neurotrophic ulceration ranges from weeks to decades, with a median of 1–7 years. 1,2 More than 80% of the reported cases of TTS develop ulceration following the dermatomal distribution of a branch of the maxillary nerve, the infraorbital nerve. The area most frequently affected is the nasal ala, which is supplied by one of the terminal cutaneous branches of the infraorbital nerve, the external nasal branch.…”

“…1,3 These findings also rule out other causes of non-healing ulcers. 1,2 Immunofluorescence tests can also be of help. 11 Treatment of TTS should be centred on behavioural modification intended to reduce self-induced trauma.…”

Trigeminal Trophic Syndrome Secondary to Refractory Trigeminal Neuralgia Treated with CyberKnife® Radiosurgery

“…It can develop due to any type of involvement of the trigeminal nerve or other causes such as central and/or peripheral alterations or functional, ischemic, traumatic, or iatrogenic etiology. Brain stem infarcts, vestibular nerve schwannomas, meningiomas and gliomas, syringobulbia, trigeminal neuralgia and its therapeutic procedures, infectious causes due to mycobacteria and herpes viruses, as well as trauma, and radiotherapy procedures have been reported as etiology [1,3,10].…”

“…Radiosurgical rhizotomy with CyberKnife has been reported as a safe and effective procedure in the treatment of trigeminal neuralgia refractory to conventional treatments and produces necrosis, mainly in the sensory fibers of the trigeminal nerve, cutting the pain circuit [10][11][12]. Ulcerations in the nasal region have been studied by different ear-nose-throat (ENT) and dermatology specialists without having been able to clarify the pathophysiology or the best way of treatment [13][14].…”