Trigeminal Trophic Syndrome: A Case Report

Case Report

Austin J Anesthesia and Analgesia. 2017; 5(2): 1060.

Trigeminal Trophic Syndrome: A Case Report

Fleischer BC¹, Pergolizzi JV¹*, Papagallo M², Taylor R¹ and Jo Ann LeQuang BA¹

¹NEMA Research, USA

²Albert Einstein Medical College, USA

*Corresponding author: Pergolizzi JV, NEMA Research, USA

Received: October 09, 2017; Accepted: October 24, 2017; Published: October 31, 2017


Trigeminal Trophic Syndrome (TTS) is a rare neurological condition that occurs as a result of a lesion affecting the peripheral or central nervous system trigeminal pathway, such as might occur following an injury to the trigeminal ganglion [1]. Cases have also been reported following neurolysis of the Gasserian ganglion for the treatment of trigeminal neuralgia [1,2]. The overall pathogenesis of TTS is not well understood. In about a third of published cases, the patient had a history of stroke; other known associations include craniotomy, head trauma, and herpes zoster manifestation [2]. The mean age at presentation of TTS is 57 years (range 1 to 93 years) [1].

The first published reports of TTS appeared in 1933 by Wallenberg and Loveman. In one case, a “carcinomatous-like ulcer” appeared on the patient’s forehead following removal of the Gasserian ganglion. In the other case, the patient suffered from persistent facial ulcers with tissue damage to the ala nasi following ipsilateral neurolysis of the Gasserian ganglion for treatment of trigeminal neuralgia [3,4].

The classic presentation for clinical diagnosis of TTS is a triad of three symptoms: anesthesia, paresthesias, and skin ulcers within the trigeminal distribution. TTS is a diagnosis of exclusion with a differential based on clinical conditions, including malignancy, Wegener granulomatosis, infections, brown recluse spider bite, pyoderma gangrenosum, and factitious dermatitis. Differential diagnosis may require biopsy [5,6]. There is no specific diagnostic algorithm. Clinicians should look for prior history or cerebrovascular accident as these are common causations.

Facial ulcerations concentrate around the ala nasi [1,2], but TTS patients tend to feel facial paresthesias rather than facial pain. However, facial pain may be reported [7]. Patients may scratch, pick, or rub the areas of the face where they feel anesthesia or sensations described as burning, itching, or tingling. Compulsively picking the affected area leads to tissue injury and ulcers. Ulcers can be very persistent and progress soon after they appear, often as a result of the patient’s continuing self-injury [1,4,8]. Over time, the ulcers can damage the trigeminal nerve either by direct injury or by impeding the blood supply [9] in a course that varies from two weeks to 30 years, with a mean time of presentation around one or two years [1,2].

The following report presents a peculiar case of TTS in a patient who had been suffering from skin ulcers for eight years.

Case Presentation

A 48-year-old Caucasian female presented with an eight-year history of a persistent non-healing ulceration of the left cheek and chin. Her past medical history included intermittent cystic acne, which initially complicated the diagnosis. She also had a history of trigeminal neuralgia that was managed with bite splints, an orthodontic treatment that relieves pain and tension in the patient’s face. Two months after starting her orthodontic treatment, a large nodular lesion appeared on the patient’s left cheek. An initial culture of the lesion did not reveal any underlying cause. She also tested negative for Methicillin-Resistant Staphylococcus Aureus (MRSA).

The first attempt at treating the ulcer with an injection of triamcinolone acetonide was unsuccessful. Then the patient underwent a series of hyaluronic acid dermal filler injections which healed the ulcer over the course of six months, but left a large hard nodule under the patient’s skin along with noticeable hypopigmentation of the area.

The lesion was painless and pruritic. The patient experienced a relentless biting and stinging sensation, which caused her to pick and scratch. A tissue biopsy was unrevealing. Parasitic etiologies were excluded. The patient began to experience depression as the lesion interfered with her personal life and undermined her well-being. She was prescribed an antidepressant which she took for 10 months without improvement. She started to feel better upon discontinuation of the antidepressant. Over two years, two partially successful surgical attempts were made to remove the lesion. The lesion healed after surgery, but a prominent subcutaneous mass could still be felt at the lesion’s superior border.

Over the next year, the lesion fully ulcerated once more. At this point, the patient stated that the scratching and picking had gotten out of control. This lesion was different from any acne lesion the patient had ever experienced in the past. Repeated tissue analysis of the lesion excluded silicone or any other chemical substance or foreign body as possible cause. Skin sensitivity testing for allergy was performed on the patient with the result that positive skin sensitivity to some dental materials was determined. These dental materials were removed, but no improvement of the lesion was observed (Figures 1-4).