Sir,

Trigeminal trophic syndrome (TTS) is a rare, self-induced, pathological process characterized by skin ulcerations appearing on dermatomes innervated by the trigeminal nerve following its damage at any level of its course [1]. It typically involves the ulceration of the nasal ala and less frequently of the cheek, upper lip, forehead, palate, and jaw [2,3]. It predominates in females, with an average age of 57 years [4]. The latency period between nerve damage and ulceration ranges from several weeks to several decades, with an average of one year [1,5]. The etiologies are numerous: iatrogenic, organic, infectious, traumatic, and idiopathic [5,6]. It is clinically characterized by the following triad: anesthesia in the sensory territory of the trigeminal, facial paresthesia (burning, tingling, numbness, tingling), and the classic crescent-shaped ulceration of the nasal ala [1,4,5,7]. The ulcerations may be single or multiple, typically unilateral, often on the right side of the face and uniform in appearance [1,4]. They are persistent due to painless, repetitive self-manipulation of the affected area [1]. Histology is necessary to exclude other causes of the facial ulceration [1,5]. The diagnosis is based on a combination of anamnestic and clinical findings and non-specific histology revealing no evidence of malignancy or infection [1]. Differential diagnoses include other causes of chronic facial ulcerations, such as infectious causes (leprosy, syphilis, blastomycosis, paracoccidioidomycosis, leishmaniasis), vasculitis (Wegener’s granulomatosis), malignancy (carcinoma, sarcoma, malignant lymphoma), destructive lethal midline granuloma, pyoderma gangrenosum, and factitial dermatitis [1,2,5].

The treatment of trigeminal trophic syndrome is often difficult and multidisciplinary. Patient education is crucial, as the self-inflicted nature of the pathology must be explained [2,5] to avoid destructive scratching habits, to apply occlusive covering, especially at night, reducing trauma and promoting healing [1]. Several therapeutic means are employed such as carbamazepine, gabapentin, pregabalin, amitriptyline, and pimozide, which reduce paresthesia [1,2,4,5,7], although none of them have proven to be permanently effective [6]. Other treatments, reported in isolated cases, with encouraging results are transcutaneous electrical stimulation, keratinocyte culture, and negative pressure therapy [4]. Finally, surgery may be proposed if the damage to the nose is highly mutilating, with skin and cartilage grafts [1,2]. Long-term follow-up is essential, as a recurrence of the ulceration is possible due to persistent trigeminal nerve damage.

Herein, we report the case of a 69-year-old female with a history of a stroke eleven months before her consultation with persistent sequelae numbness in the right hemiface. She consulted for asymptomatic lesions of the face evolving for six months. An examination revealed two ulcerations with clean surfaces, surmounted by hemorrhagic crusts, with the largest measuring 1.2 cm, with a non-infiltrated base opposite to the right nasolabial fold, with partial destruction of the non-cartilaginous right nasal ala, and with hyper-pigmented peri-lesional skin (Fig. 1a). Dermoscopy revealed an ulceration and whitish structures in the periphery without any particular vascularization. A neurological examination revealed a thermoalgesic anesthesia of the right hemiface. The rest of the somatic examination was unremarkable. A biopsy was taken from both lesions, and the anatomopathological study was non-specific, eliminating a malignant or infectious etiology. The interrogation was repeated with the patient who recognized that the lesions were the result of iterative rubbing of the affected areas because of persistent paresthesia. Trigeminal trophic syndrome was retained. The patient was treated with gabapentin 600 mg per day for two months with a considerable improvement in the paresthesia. The patient was able to refrain from scratching and apply healing cream to the ulcerations, with the quasi-total disappearance of the ulcerations (Fig. 1b).

Figure 1: (a) Two ulcerations opposite to the nasolabial fold with the partial destruction of the non-cartilaginous right nasal ala. (b) Two months after treatment with resolved ulcerations yet persistent loss of the non-cartilaginous right nasal ala.

Consent

The examination of the patient was conducted according to the principles of the Declaration of Helsinki.

REFERENCES

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