Trigeminal trophic syndrome: A report of two cases with review of literature
Dermatology Department, EPS Habib Thameur, Tunis. email@example.com
Trigeminal trophic syndrome, a rare cause of facial ulceration, is the consequence of damage to the trigeminal nerve or its central sensory connections. We report two cases of trigeminal trophic syndrome, complications of cerebral vascular accidents. A 65-year-old woman complained of 2 months of numerous and progressive ulcers of the right side of her face. A 67-year-old woman presented with an ulcer of the ala nasi for 10 weeks. This syndrome most commonly occurs in women with an average age of 57 years. About 100 cases are reported in the literature. The diagnosis is made on clinical grounds and is suggested when loss of sensation occurs in association with unilateral facial ulceration, especially involving the ala nasi. Once the ulcers appear, they are extremely persistent.
L'ulcération neurotrophique trigeminée est une pathologie rare, compliquant l'atteinte du nerf trijumeau ou une de ses branches centrales. Nous rapportons 2 cas d' ulcération neurotrophic trigeminée survenant après un accident vasculaire cérébral. La première concerne une femme âgée de 65 ans, présentant depuis 2 mois une ulcération de l'aile droite du nez, du canthus interne droit et de la tête du sourcil. La 2ème observation intéresse une femme âgée de 67 ans, consulte pour une ulcération arciforme de l'aile du nez évoluant depuis 2 mois. Une centaine de syndrome neurotrophique est rapporté dans la littérature. Il atteint préférentiellement la femme avec un âge moyen de 57 ans. Le diagnostic est clinique posé sur l'hypoesthésie du territoire du trijumeau associé à une ou plusieurs ulcérations unilatérales torpides localisées essentiellement à l'aile du nez.
Trigeminal trophic syndrome was first described by Wallenberg in 1901 as an uncommon clinical entity in which cutaneous trophic ulceration develops within trigeminal dermatomes . We report two cases of trigeminal trophic syndrome complicating cerebral vascular accidents. These cases illustrate the diagnosis and management problems presented by this syndrome.
First case.A 65-year-old woman complained of numerous progressive ulcers of the right side of her face for 2 months. She had a past medical history of a cerebral vascular accident 2 years before. The ulcers were painless and were located on ala nasi, right canthus, tip of the eyebrow, and the forehead. Their margins were clearly delineated (fig.1). Neurological examination revealed decreased level of pain and thermal perception over the trigeminal area as well as a diminution of corneal and mastication reflexes. Histology was not done. Syphilis serology was negative. The patient was diagnosed with neurotrophic ulcer.
Second case.A 67-year-old woman presented with an ulcer of the ala nasi for 10 weeks. She had hemiparesthesia of the face with reflex itching. She had suffered a cerebral vascular accident one year before. The ulcer was painless, was located at ala nasi, and had the characteristic sickle-shape defect. The lesion had scant crust, and the margins were clearly delineated (fig. 2). A biopsy of the lesion revealed reactive hyperplasia of the dermis with neither malignant-cellular proliferation nor granulomatous infiltration. Neurological examination revealed decreased perception of light touch and pain sensation over the trigeminal area. The diagnosis of neurotrophic ulcer was confirmed.
Trigeminal trophic syndrome is a rare consequence of damage to the trigeminal nerve or its central sensory connection . There are 100 cases reported as of 1998 . This syndrome has a predilection for women and occurs at an average age of 57 . 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 . Patients complain of picking, rubbing, or scratching sensations in the affected areas; these sensations are attributed to hypsesthesia, paraesthesia, or pain following damage of the sensory trigeminal fibers or nuclei. There may be a delay in recognizing the association of a unilateral ulcer on the trigeminal area (first and second branch of trigeminal nerve), with the underlying nerve damage. Often, the ala nasi is involved with a characteristic painless, sickle-shaped lesion. The margins are free, and the ulcer base has scant crust. Other areas may be involved with these ulcers, including the frontal scalp (resulting in triangular frontoparietal alopecia), ear, cheek, temple, and cornea . Once the ulcers appear, they are extremely persistent. Rare cases of self-healing in the trigeminal dermatome are reported [7, 8].
Trigeminal trophic syndrome occurs after neurosurgical intervention in 75 percent of cases, 70 percent involve surgical removal of the Gasserian ganglion. Other associations include Wallenberg syndrome (6 %), and following a cerebral vascular accident (7 %). Exceptionally, these lesions may occur following encephalitis, meningioma, syringobulbia, or during tabes .
The diagnosis is clinical and is suggested when loss of sensation occurs in association with unilateral facial ulceration, especially involving the ala nasi. 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 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. 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 because these agents may influence both the paresthesias and behavioral factors in this syndrome .
Trigeminal trophic ulceration is a rare diagnosis that classically complicates surgical procedures meant to alleviate essential neuralgias of trigeminal nerve. It has been described in association with conditions such as cerebral vascular accidents, as is the case for our two patients. The diagnosis is clinical and a histological exam helps to rule out other entities in the differential diagnoses. Once the ulcers appear they are extremely persistent.
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