Trigeminal Trophic Syndrome

 Updated 3/2017-- photos and all links removed as many no longer active.

I don't recall ever reading about this syndrome or seeing a patient with it. The picture of the patient in the January 2008 (first reference) case presentation caught my eye. I was drawn in and wanted to know more about trigeminal trophic syndrome (TTS). I thought I would share it with you.

It seems that TTS was first described by Wallenberg in 1901. It was described as an uncommon clinical entity in which cutaneous trophic ulceration develops within trigeminal dermatomes. The trigeminal nerve (CN V) is primarily a sensory nerve and is responsible for sensation in the face.

TTS is a rare consequence of damage to the trigeminal nerve itself or its central sensory connection. The average period from the time of trigeminal nerve injury to the onset of the ulcer varies from several weeks to many years. Patients complain of picking, rubbing, or scratching sensations in the affected areas. These sensations are attributed to hypoesthesia, paraesthesia, or pain following the nerve damage. Conditions that can affect the trigeminal nerve include brainstem cerebrovascular disease, diabetes, tabes, syringomyelia, and postencephalopathic parkinsonism. It can also occur following surgical management of trigeminal neuralgia.

The diagnosis is clinical. It is suggested when loss of sensation occurs in association with unilateral facial ulceration, especially the nasal ala. Patients tend to complain of paresthesias and painless traumatization.

The differential diagnosis includes basal cell carcinoma, infectious diseases (tertiary syphilis, recurrent herpes simplex, leishmaniasis, cutaneous tuberculosis, or leprosy) or systemic disease (Wegener granulomatosis, Horton disease). Similar lesions may occur with factitial ulcers, but the psychiatric context is different.

The characteristic lesion is a painless, sickle-shaped lesion involving the nasal ala. The margins are free. The ulcer base has scant crust. Other areas that may be included with these ulcers include the frontal scalp, ear, cheek, temple, and cornea. Once the ulcers appear, they are very persistent. Neurological examination will reveal decreased sensation of pain and thermal perception over the trigeminal area (very important for making the diagnosis).

Management of trigeminal trophic syndrome is often difficult. Application of occlusive dressings can reduce trauma of the affected area. Hydrocolloid dressings help the ulcers heal. Treatment regimens involving amitriptyline and diazepam in addition to chlorpromazine and pimozide have met with some success. Carbamazepine is an effective therapy in trigeminal neuralgia and atypical facial pain because these agents may influence both the paresthesias and behavioral factors in this syndrome.

Surgical repair is usually unsuccessful. Surgical repair may be worthwhile, however, if tissue with functional innervation and its own blood supply is used.

Didn't share the picture from the first reference (the one that caught my attention). In it, the entire ala of the nose and more was missing. It is similar to the one that can be found here (be cautioned -- it's graphic).

This is not a joke post. I love jokes, but am not good at telling them. I say this as it is April Fool's Day! There are others who are good at pulling off good jokes -- check here and here.

REFERENCES

Trigeminal Trophic Syndrome: Diagnosis and Management Difficulties; Plastic & Reconstructive Surgery. 121(1):1e-3e, January 2008; Bhatti, Ahmad F. F.R.C.S.I., A.F.R.C.S.I.; Soggiu, Daniela M.D.; Orlando, Antonio F.R.C.S., Dip.E.B.O.P.R.A.S.

Skin Ulceration in Trigeminal Trophic Syndrome: Report of a Lesion Occurring 22 Years Later; Plastic & Reconstructive Surgery. 116(6):1814-1815, November 2005; Yiacoumettis, Andreas M.D., Ph.D.; Vlachos, Spiros M.D., Ph.D.

Trigeminal Trophic Syndrome: A Report of Two Cases with Review of Literature; Dermatology Online Journal 9(5):26; A Elloumi-Jellouli, S Ben Ammar, S Fenniche, M Zghal, H Marrak, and I Mokhtar

Trigeminal Trophic Syndrome; Mayo Clin Proc 1997; 72:543-545; Charles H Dicken MD