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Last updated: 3/07/2024
Years published: 2024
NORD gratefully acknowledges Lauren Walters and Nikita Baral, MD candidates, Creighton University School of Medicine, and Ryan Trowbridge, MD, MS, MA, FAAD, Associate Professor of Dermatology, Creighton School of Medicine, for the preparation of this report.
Summary
Trigeminal trophic syndrome (TTS) is a rare condition caused by an injury to the trigeminal nerve (fifth cranial nerve). Symptoms include loss of sensation or uncomfortable sensations on the skin of the face and ulceration of the affected skin. Patients may feel numbness, itching or burning that leads to scratching and rubbing the skin and eventually open sores (ulcers). Ulcers are slow to heal or non-healing. Symptoms are limited to one side of the face, most commonly the outside of the nostril, though the eyelid, forehead, scalp, cheek or lips can also be affected. Diagnosis is clinical, meaning there are no tests that need to be performed. However, a doctor may need to rule out other causes of ulceration, such as cancerous or infectious lesions.
Introduction
The trigeminal nerve provides sensation to the face and can be divided into the ophthalmic, maxillary and mandibular branches which supply nerves to the top, middle and bottom of the face, respectively. TTS occurs after the trigeminal nerve has been injured. There are many causes of nerve injury, including but not limited to stroke, infection or prior surgery in the surrounding area.
The following symptoms may develop in the areas of the face affected by TTS:
Changes in sensation lead to rubbing and scratching the skin, which usually results in a crescent shaped ulceration on the side of the nostril. Ulceration commonly begins at the nose and extends down the cheek to the upper lip, resulting in a sneer-like appearance. Less commonly, the eyelid, forehead and scalp may be affected.
TTS occurs after injury or damage to the trigeminal nerve. Symptoms can appear weeks to years after injury.
Reported causes of damage to the trigeminal nerve leading to TTS include:
Trigeminal trophic syndrome is an extremely rare diagnosis. Females are more often affected than males. TSS is more common in middle to late adulthood, but younger people are also diagnosed with this syndrome. The average age of diagnosis is 57 years. Until 2019, there were about 200 patients reported in the medical literature.
Clinical Testing and Work-up
Diagnosis of TTS is clinical, meaning it is based on the patient’s medical history and symptoms. A history of neurologic diseases like stroke or shingles in the affected area makes the diagnosis more likely. The doctor may swab the area and send it to a lab to rule out infection. A biopsy of the affected skin may also be obtained to rule out cancerous or inflammatory lesions.
Treatment
There is no cure or widely accepted standard therapies for TTS. However, there are a variety of treatments that can be used to improve symptoms.
The cornerstone of treatment is to reduce behaviors that cause ulceration. Patients need to learn to avoid rubbing or scratching the skin. Other therapies will be more effective if the patient can do this.
Coverings like silicone sheets or bandages can be used to cover the areas of ulceration to prevent further damage and allow healing. Medical therapy may include medications such as gabapentin, amitriptyline or carbamazepine that all work at the level of the nerve to decrease uncomfortable sensations. Treatment with a mild electrical current called transcutaneous nerve stimulation has also been shown to be somewhat helpful.
In cases that fail to respond to these treatments, surgery may be an option. Using a flap of skin from an unaffected part of the face to cover the affected area has been successful in some patients.
The combination of skin breakdown and rubbing and scratching increases the risk for infection. If symptoms suddenly worsen or spread, patients should be seen by a doctor to rule out a new infection. For cases that affect the area around the eyes, frequent follow up with an ophthalmologist is recommended because infections affecting the eyes can affect vision.
Information on current clinical trials is posted on the Internet at https://clinicaltrials.gov/ All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government website.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]
Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/
For information about clinical trials sponsored by private sources, contact:
https://www.centerwatch.com/
For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/
JOURNAL ARTICLES
Rybak-D Obyrn J, Alicja LJ, Joanna MN, Agnieszka OS, Waldemar P. Trigeminal trophic syndrome associated with neuralgia after ischemic stroke-a case report. Clin Case Rep. 2023;11(8):e7711. Published 2023 Aug 7. doi:10.1002/ccr3.7711
Tehfi D, Barrera-Godínez A, Dominguez-Cherit J, Gatica-Torres M. Trigeminal trophic syndrome as a complication of herpes zoster ophthalmicus. Cureus. 2022;14(10):e30382. Published 2022 Oct 17. doi:10.7759/cureus.30382
Lee DW, Ryu HR, Choi HJ, Kim JH. One-stage reconstruction of full-thickness alar defects with a folded nasolabial island flap. Arch Craniofac Surg. 2021;22(6):296-302. doi:10.7181/acfs.2021.00339
Carr P, Martin S, Young J, Chiota-McCollum N. Trigeminal trophic syndrome: A possible dermatologic manifestation of stroke deficits. Neurol Clin Pract. 2020;10(4):e27-e29. doi:10.1212/CPJ.0000000000000711
Bradburn KH, Elston D, Murphey AW, Patel KG. Trigeminal trophic syndrome—A unique clinical presentation of a rare condition. Ear, Nose & Throat Journal. 2019;98(10):606-608. doi:10.1177/0145561319850818
Khan AU, Khachemoune A. Trigeminal trophic syndrome: an updated review. Int J Dermatol. 2019 May;58(5):530-537. https://pubmed.ncbi.nlm.nih.gov/29931799/
Thompson LB, Powell SL. Trigeminal trophic syndrome leading to orbital cellulitis. Clin Pract Cases Emerg Med. 2018;2(2):121-124. Published 2018 Mar 14. doi:10.5811/cpcem.2018.1.36622
Palanisamy A, Rajappavu SD, Kothandapany S. Trigeminal trophic syndrome. An Bras Dermatol. 2017;92(4):593-594. doi:10.1590/abd1806-4841.20175484
Luksić I, Luksić I, Sestan-Crnek S, Virag M, Macan D. Trigeminal trophic syndrome of all three nerve branches: an underrecognized complication after brain surgery. J Neurosurg. 2008;108(1):170-173. doi:10.3171/JNS/2008/108/01/0170
Dicken CH. Trigeminal trophic syndrome. Mayo Clin Proc. 1997;72(6):543-545. doi:10.4065/72.6.543
Finlay AY. Trigeminal trophic syndrome. Arch Dermatol. 1979;115(9):1118.
INTERNET
Amerasinghe N. Trigeminal Trophic Syndrome. DermNet.2023. https://dermnetnz.org/topics/trigeminal-trophic-syndrome Accessed Dec 19, 2023.
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