Trigeminal trophic syndrome (TTS) is a rare condition characterized by cutaneous ulceration in the distribution of the trigeminal nerve, most commonly the infraorbital nerve, due to self-inflicted trauma from facial dysesthesia. It results from damage to the trigeminal nerve pathway from various etiologies like iatrogenic nerve injury, cerebrovascular disease, or malignancy. Clinically, patients present with trigeminal anesthesia, facial paresthesia, and crescent-shaped nasal ulcers. Treatment is multidisciplinary and includes behavioral modification, neurologic evaluation, pain management, and various medical therapies.

1. Trigeminal Trophic Syndrome
Ade Wijaya, MD – September 2023

2. Outline:
• Etiopathogenesis
• Etiology
• Clinical Presentation
• Workup
• Differential Diagnosis
• Management
• Summary

3. Etiopathogenesis
• Uncommon cause of cutaneous ulcers in the trigeminal
dermatome, most often involving the nasal ala
• Results from damage to the central or peripheral
components of the trigeminal nerve pathway leading to
facial dysesthesia and trigeminal hypoesthesia or
anesthesia.
• Self-inflicted manipulation and trauma to the dysesthetic
skin leads to cutaneous injury and ulcer development
• More than 80% of the reported cases develop ulceration
in the distribution of a branch of the maxillary nerve, the
infraorbital nerve
Bradburn KH, Elston D, Murphey AW, Patel KG. Trigeminal trophic syndrome—a unique clinical presentation of a rare condition. Ear, Nose & Throat Journal. 2019
Dec;98(10):606-8.

4. Etiology
• Iatrogenic damage (eg, rhizotomy) to the
trigeminal nerve.
• Other causes include cerebrovascular disease,
malignancy (eg, astrocytoma, acoustic neuroma,
intracranial meningioma), herpes zoster virus,
postencephalitic parkinsonism, syringobulbia,
and trauma.
• Cutaneous manifestations can appear 2 weeks to
30 years after nerve insult
Bradburn KH, Elston D, Murphey AW, Patel KG. Trigeminal trophic syndrome—a unique clinical presentation of a rare condition. Ear, Nose & Throat Journal. 2019
Dec;98(10):606-8.

5. Clinical Presentation
Trigeminal anesthesia
Facial paresthesia
Crescent-shaped ulceration
of the lateral nasal ala
Bradburn KH, Elston D, Murphey AW, Patel KG. Trigeminal trophic syndrome—a unique clinical presentation of a rare condition. Ear, Nose & Throat Journal. 2019
Dec;98(10):606-8.

6. Workup
• Clinical history, neurological examination,
serology, and biopsy of the lesion to rule out
other causes of nonhealing ulcers
Rashid RM, Khachemoune A. Trigeminal trophic syndrome. Journal of the European Academy of Dermatology and Venereology. 2007 Jul;21(6):725-31.
Lukšić I, Lukšić I, Šestan-Crnek S, Virag M, Macan D. Trigeminal trophic syndrome of all three nerve branches: an underrecognized complication after brain surgery: Case report.
Journal of neurosurgery. 2008 Jan 1;108(1):170-3.

7. Differential Diagnosis
• Neoplasms (basal cell and squamous cell
carcinoma, lymphoma)
• Cutaneous vasculitis
• Infectious processes (herpetic ulcer, leprosy,
syphilis, mucormycosis)
• Granulomatosis with polyangiitis
• Pyoderma gangrenosum
Bradburn KH, Elston D, Murphey AW, Patel KG. Trigeminal trophic syndrome—a unique clinical presentation of a rare condition. Ear, Nose & Throat Journal. 2019
Dec;98(10):606-8.

8. Management
• Multidisciplinary including behavioral
modification, neurologic evaluation, pain
management, medical treatment, surgical repair,
and psychological management
• Medical management includes carbamazepine,
pregabalin, gabapentin, amitriptyline, and
pimozide
• Transcutaneous electrical stimulation has also
been studied in single case reports and aims to
improve blood supply and healing of the area

9. Summary
• Rare condition
• Most commonly encountered in the infraorbital
nerve distribution, this case demonstrates that
ulcerations caused by TTS can occur at any
sensory branch of the trigeminal nerve
• When properly diagnosed, can avoid
unnecessary costly workups and delay in proper
treatment