Trigeminal Trophic Syndrome

  • Ellen H. de Moll, MD, Sandra Wainwright, MD, and Charles Halasz, MD
  • Volume 08 - Issue 3

Trigeminal trophic syndrome (TTS) is defined by characteristic crescentic ulcers, predominantly of the nasal ala, extending to cheek and upper lip, which can also develop on the forehead and scalp with an associated sense of itch, formication or burning on the face. It is typically associated with damage to the trigeminal nerve.

Case Report

A 48-year-old Caucasian man with a past medical history notable for obesity, peripheral neuropathy, right lateral medullary stroke 11 months prior to presentation, hypertension, diabetes, hyperlipidemia, atrial fibrillation, and depression, presented to the dermatology clinic with a right infranasal ulceration and complaints of facial “tingling” (Figure 1).

A wound culture from May 2013 grew Staphylococcus aureus, and the patient used a combination gel composed of 2.0 percent hydrocortisone acetate, 1.0 percent iodoquinol and 1.0 percent aloe polysaccharides. Viral culture was negative for herpes simplex virus (HSV) 1 and 2. A curettage biopsy was performed without complication and demonstrated broad areas of erosion covered by fibrin and neutrophils. The dermis demonstrated increased vascularity and neutrophilic and lymphocytic infiltrate. Despite treatment with antibiotic cream, the patient complained of a persistent tingling sensation and was referred to neurology.

He was evaluated by neurology and started on gabapentin with significant improvement of facial tingling.

The patient was referred to wound care by his dermatologist for a second opinion on wound management. The patient was using hyaluronic acid gel, but the wound on examination was desiccated with dried fibrin at the ulcer base. Additionally, the patient admitted that he felt compelled to touch the wound throughout the day, in part by habit and in part due to the underlying tingling sensation.

The decision was made to apply a hydrocolloid dressing to provide warmth, moisture and an occlusive barrier that would protect the wound from the patient. The patient was seen the following week to assess response to therapy. With hydrocolloid dressing, gradual resolution of the ulcer was seen, and patient had total healing within six weeks of being seen in wound care (Figure 1).

Discussion

The true epidemiology of TTS is not known, though its association with stroke, as in this patient, and other less-common causes is well described, but it is often misdiagnosed and underreported. There has been growing evidence in both neurology and dermatology literature that points to the relative frequency of this disease.

The development of TTS has been associated commonly with stroke (30 percent), including Wallenberg syndrome.¹ Other causes of TTS include trigeminal nerve ablation (30 percent), craniofacial surgery (21 percent) and less commonly neuroma, postencephalitic parkinsonism, syringobulbia, meningioma and astrocytoma.²

In one review of 60 cases of TTS, the nasal ala were involved in 79 percent of cases, the cheek 28 percent and corneal lesions 18 percent.3 The differential includes malignancy and multiple infectious causes (Table 1). Biopsy is often nonspecific but may be helpful in ruling out malignancy and special stains to rule out less-common infections. In some cases, the pathology has shown a decrease in sensory nerves in affected skin.3,4

Although treatment regimens vary widely, most practitioners agree that patient education and behavior modification are crucial to the success of therapy.5

Hydrocolloid dressing has reportedly been effective in a seven patient case series, as it was in our patient.2 Hydrocolloid is beneficial as it serves as an anatomic barrier as well as a physical barrier that prevents further manipulation of the wound by patients inadvertently due to underlying irritant sensation. In general, surgical repair is often unsuccessful.2

FIGURE 1. Before hydrocolloid dressing and gabapentin

FIGURE-1-

 

 

 

FIGURE 2. After hydrocolliod dressing and gabapentin

FIGURE-2

Systemic therapy is often successful when there is underlying burning or tingling sensation, to help limit patient’s desire to irritate the wound further. Gabapentin has been shown to be effective as a first-line treatment, with carbamazepine demonstrating somewhat less efficacy as a second-line therapy.³ There is less evidence to support the use of pregabalin, amitryptiline, pimozide given the small number of published cases. Essentially, TTS is a disorder in which neuropathy stimulates the patient to touch the wound. Blunting the neuropathic sensation with medication and employing a dressing that acts as a barrier and is cosmetically acceptable like a hydrocolloid were the successful therapies in this case.

TABLE 1
Infectious Other
Herpes Pyoderma gangrenosum
Syphilis Wegner's granulomatosis
Yaws Midline granulomatosis
Leprous trigeminal neuritis Factitial dermatitis
Deep fungus  
Mycobacteria  
Rhinoscleroma  
Leishmaniasis  

 

TTS can pose a diagnostic and therapeutic dilemma to physicians in multiple specialties, and, as in our case, patients may benefit from multidisciplinary management to address the dermatology/wound and neurologic manifestations of their disease. It is crucial to consider TTS in the differential for patients with unilateral facial ulcers, particularly with history including stroke or trigeminal nerve ablation.

Appropriate education and therapy can lead to greatly improved outcomes and full healing of the ulcer.

 

References

  1. Goodnight JW, Calcaterra T. Trigeminal trophic syndrome: a report of two cases and review." American Journal of Otolaryngology. 1994; 15.3: 219-22.

  2. Dicken CH. Trigeminal trophic syndrome. Mayo Clinic Proceedings. 1997; 72.6: 543-45.

  3. Sawada T. Asai J. Nomivama T, Masuda K, Takenaka H, Katoh N. Trigeminal trophic syndrome: report of a case and review of the published work. The Journal of Dermatology. 2014; 41:525-28.

  4. Dolohanty LB, Richardson SJ, Herrmann DN, Markman J, Mercurio MG. Trigeminal trophic syndrome with histopathologic correlation. Cutis. 2015; 95: E22-25.

  5. Pichard, DC, Cowen EW. Trigeminal trophic syndrome after stroke. Mayo Clinic Proceedings. 2014; 89.9: e87-88.

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