Trigeminal trophic syndrome.

Trigeminal trophic syndrome (TTS) is a rare cause of facial ulceration, consequent to damage to the trigeminal nerve or its central sensory connections. We reporta case of TTS in a 48-year-old woman with Bell's palsy following herpes zoster infection. The patient was treated and counseled. There hasnot been any recurrence for 1 year and the patient is being followed-up. The diagnosis of TTS should be suspected when there is unilateral facial ulceration, especially involving the ala nasi associated with sensory impairment.

What was known?

TTS is known to occur following, Hansen's disease and herpes zoster involving the trigeminal nerve.

Introduction

Trigeminal trophic syndrome (TTS) is a rare condition resulting from self-manipulation of the skin after a peripheral or central injury to the trigeminal system. The syndrome consists of a triad of anesthesia, paresthesia, and a secondary persistent or recurrent facial ulceration.[1] It was first described as a cutaneous ulceration in the trigeminal dermatome by Wallenberg, in 1901, and later by Loveman in 1933.[23] The common dermatological causes are Hansen's disease and herpes zoster. We report a case of TTS following herpes zoster in a 48-year-old woman with Bell's palsy.

Case Report

A 48-year-old woman complained of nonhealing painless progressive ulcer over the left nostril and skin over the upper lip of 6-months duration. She had herpes zoster affecting the left side of face 1-year back. For the facial ulcer, she was treated with both topical and systemic steroid and multiple antibiotics without any improvement. The ulcers were painless located on ala nasi, extending medially into the nasal septum, superiorly over the skin of the nose and inferiorly involving the skin over the upper lip on the left side. Their margins were clearly delineated [Figure 1].

Figure 1

Case of treatment of trigeminal trophic syndrome showing ulcer involving the ala nasi on the left side with extension to the surrounding skin and mucosa

There were no vesicles around the ulcer or anywhere over the face. There were no hypopigmented patch or peripheral nerve thickening. Neurological examination revealed left-sided lower motor neuron type of facial palsy, decreased level of pain, and temperature sensation over the trigeminal area. Corneal and mastication reflexes were diminished. Her vitals and other systemic examination revealed no abnormality. Tzanck smear from the ulcer showed neutrophils and there were no acantholytic or multinucleate giant cells. Touch smear for bacteria and slit skin smear for acid fast bacilli were negative. Antinuclear, antineutrophil cytoplasmic; hepatitis B and C, and antibodies were negative. Tests for HIV and syphilis were nonreactive. Her blood glucose, renal, and liver function tests were within normal range. Electrocardiography (ECG) and chest radiography, computed tomography (CT) scan, and magnetic resonance imaging (MRI) of the brain were normal.

TTS was diagnosed and the ulcer was treated with saline soaks and topical antibiotics. Patient was counseled and advised to avoid scratching or rubbing the site. There was remarkable improvement and the ulcer healed in 2 weeks time [Figure 2]. Patient is being followed-up and there is no recurrence since 1 year.

Figure 2

Trigeminal trophic syndrome Ulcer healing with scarring

Discussion

TTS is a rare consequence of damage to the trigeminal nerve or its central sensory connection.[4] TTS is more common in women than men.[5] TTS presents following certain conditions and diseases like; trigeminal nerve ablation, Wallenberg syndrome (stroke/vascular insufficiency), tumor, postencephalitic sequelae, trauma, craniotomy, amyloid deposits in the CNS and trigeminal nerve, herpes zoster, herpes simplex, syphilis, and neurological complications from birth trauma or it can be idiopathic.[1] There are reports of TTS occurring in patients with leprosy and the authors of this correspondence have described the same asearly as in1991.[67] TTS is also common to occur following herpes zoster infection. Trigeminal dysfunction is also frequently associated in patients with Bell's palsy.[8]

The average period from the time of trigeminal nerve injury to the onset of the ulcer varies from several weeks to many years, with an average of 2 years. The earlier onset of clinical manifestation may be due to involvement of multiple cranial nerves. Chief complaints are picking, rubbing, or scratching sensations in the affected areas. Most of them present with nonhealing ulcer. Often, the ala nasi is involved with a characteristic painless, sickle-shaped lesion with a defined margin. Once the ulcers appear, they are extremely persistent. In our case with a similar presentation, the diagnosis of TTS was essentially from the history and clinical findings. TTS has to be and was differentiated from, infectious diseases (varicella zoster, recurrent herpes simplex, leprosy, tertiary syphilis leishmaniasis, cutaneous tuberculosis, blastomycosis, paracoccidioidomycosis), Wegener's granulomatosis, giant-cell arteritis, basal cell carcinoma, squamous cell carcinoma, destructive lethal midline granuloma, pyoderma gangrenosum, and temporal arteritis. Factitial ulcers will closely mimic TTS but the sensory loss seen in TTS will not be a feature in factitial dermatitis.[9]

The management of TTS is often difficult. Application of occlusive dressings can reduce trauma of the affected area. Hydrocolloid dressings help the ulcers heal. Transcutaneous electrical nerve stimulation was reported to be of use in some cases. 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. Successful treatment of TTS in a 6-year-old boy with negative pressure wound therapy has been reported.[10]

Conclusion

The diagnosis of TTS should be suspected when there is unilateral facial ulceration, especially involving the ala nasi associated with sensory impairment. High index of suspicion, awareness, and appropriate treatment will prevent further mutilation.

What is new?

TTS following herpes zoster of multiple cranial nerves is an unusual occurrence. Earlier onset of ulceration could be attributed to the multiple nerve involvement, which is so far not reported in Indian literature.