When nonspecific histology can be a clue to the diagnosis: three cases of trigeminal trophic syndrome

Trigeminal trophic syndrome (TTS) is a rare cause of facial ulceration, which is usually associated with damage to the trigeminal nerve pathway, either centrally or peripherally, the most common causes being cerebrovascular accidents and trigeminal nerve ablation procedures. We present three cases of TTS, emphasizing the histopathological features. All three patients presented with facial ulceration. Two patients had a single lesion, and the third had several ulcers. However, in all cases, there was involvement of the nasal ala, and the lesions were strictly unilateral. Histology consistently showed ulceration with signs of severe chronic trauma: scarring, lichenification and/or pseudoepitheliomatous hyperplasia. Diagnosis of TTS can be difficult, and requires close clinicopathological correlation. Histology is important in excluding the majority of possible conditions included in the differential diagnosis, mainly malignancy and infectious processes. Several treatments have been described, but TTS is frequently refractory to treatment.     

Trigeminal trophic syndrome

Trigeminal trophic syndrome (TTS) is a rare cause of facial ulceration, which is believed to develop after insult to the trigeminal ganglia or other parts of the peripheral/central nervous system in the trigeminal pathway. The pathogenesis of TTS is poorly understood. Developing a better understanding of TTS will allow early recognition and improved treatment. Although the ulcers develop predominantly on the ala nasi, the literature on ulcer locations is limited. In this article, we review the epidemiologic aspects of TTS, expand on our knowledge of the anatomic location of the ulcers, and discuss current theories for its aetiology and briefly review the approaches to its management.     

Trigeminal Trophic Syndrome from Stroke

Trigeminal trophic syndrome (TTS) is the historic name for neuropathic self-induced facial ulceration from abnormal sensory symptoms leading to uncontrolled scratching. Anatomic co-localization of sensory loss (numbness) plus neuropathic itch and pain permits painless scratching. If the itch is severe, some patients will scratch to the point of causing self-injury. Patients may be unaware or may conceal the fact that their lesions are self-induced and thus the diagnosis presents a clinical challenge. Many cases remain undiagnosed, leading to unnecessary and ineffective tests, procedures, and prescribing. We document a patient with a central cause of TTS - multiple cerebral vascular accidents - and summarize the presentation, pathogenesis, and treatment options.     

Self-induced nasal ulceration from trigeminal trophic syndrome

Trigeminal trophic syndrome is a rare entity characterized by the presence of ala nasi ulceration, trigeminal anesthesia, and paresthesia. It arises secondary to trigeminal nerve injury. Patients with this diagnosis often undergo repeated skin biopsies to rule out suspected nonmelanoma skin cancer due to the clinical appearance. Recognition of this entity is paramount to avoid unnecessary surgical intervention and to attempt therapy.     

Trigeminal trophic syndrome: a report of two cases with review of literature

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 numerous and progressive ulcers of the right side of her face of 2 month's duration. A 67-year-old woman presented with an ulcer of the ala nasi of 10 week's duration. 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.     

Trigeminal trophic syndrome: a rare entity

Trigeminal trophic syndrome 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 classic triad of anaesthesia, paraesthesia, and a secondary persistent or recurrent facial ulceration. We describe a 60 year-old woman who developed this syndrome as a sequel to the gasserian ganglion block for trigeminal neuralgia. She had also developed melasma within 1 year. A remarkable benefit was achieved by proper patient education and topical antibiotics which led to the healing of all ulcerations within 4 weeks. In the case reported here, the diagnosis of the trigeminal trophic syndrome was made primarily as a result of the physician's experience with the syndrome previously.     

Trigeminotrophe Ulzeration des Nasenflügels bei Wallenberg-Syndrom

A patient developed a trophic ulceration of the nose after an acute bulbar ischemia with infarction of the right trigeminal nuclei. Neurologic examination showed symptoms of a Wallenberg syndrome including ipsilateral hyp- and paresthesia of the second trigeminal branch and disturbed sensibility and temperature sense on the contralateral half of the body. The right ala nasi showed the characteristic sickle-shaped defect (ulcération en arc). The differential diagnosis and therapeutic approaches are discussed.     

Post-apopletic trigeminal trophic syndrome

Trigeminal trophic syndrome is an uncommon clinical entity in which cutaneous trophic ulceration develops with continuous manipulation of trigeminal dermatomes. Patients spontaneously refer picking, rubbing and/or scratching at the affected areas because of hypo-anaesthesia, paraesthesia and/or pain following damage of the sensory trigeminal fibres or nuclei. We herein describe a patient who developed the syndrome as a sequela of brain stem infarction. Diagnosis by scrape cytology in ruling-out basal cell carcinoma and other ulcerative skin diseases is discussed and the importance of neurological examination in disclosing hemi-anaesthesia of trigeminal dermatome(s) is emphasized.     

Trigeminal trophic syndrome: An unusual nasal ulceration. A case report and review of the literature

Trigeminal trophic syndrome is a rare condition characterized by self-inflicted persistent facial ulceration. It is believed to be consequent to central or peripheral insult to trigeminal nerve, which may have taken place even years before the ulcer development. The aggression to the nerve pathway causes dysesthesias in the trigeminal dermatomes that induce a self-mutilating behavior, with repetitive pinching or scratching in order to mitigate the altered sensation. Due to associated skin anesthesia, the patient does not interrupt manipulation of the affected area despite severe skin necrosis. Ulceration typically occurs in the ala nasi and may resemble other more common cutaneous diseases, such as tumors or infections. Given that this condition is not included in our daily clinical practice, the risk is that of a diagnostic delay with devastating functional and esthetic facial consequences. We present the case of a patient with a history of meningioma resection who developed this syndrome and we have reviewed the published literature to provide an update on the etiopathogenesis, diagnosis and treatment of this rare condition.     

Trigeminal neurotrophic ulceration–a report of four patients

We describe four elderly patients who developed neurotrophic ulceration in the trigeminal area of the face. In three patients, ulceration followed alcohol injection of the Gasserian ganglion for the treatment of intractable trigeminal neuralgia. In the fourth patient, ulceration in anaesthetic skin followed destruction of the sensory root of the trigeminal nerve in an area of brain stem infarction caused by occlusion of the posterior inferior cerebellar artery.     

Improvement of trigeminal neurotrophic ulceration with pimozide in a cognitively impaired elderly woman—a case report

Neurotrophic ulceration of the face is a rare but well recognized sequel to division of the trigeminal nerve. ^1,2 Trauma is an important contributory factor and thought to be due in part to paraesthesiae, which encourages picking and scratching, with resultant chronic and persistent ulceration. ³ A case is described of an 82-year-old woman with severe trigeminal neurotrophic ulceration which improved substantially with pimozide, given for treatment of unrelated paranoid symptoms. The possible relevance of this to the established use of pimozide in delusional parasitosis is briefly discussed.     

Trigeminal trophic syndrome: Report of a case and review of the published work

Trigeminal trophic syndrome is a rare complication of trigeminal nerve injury that causes facial ulceration, anesthesia and paresthesia in the same trigeminal dermatomes. We present a case of a 65‐year‐old woman with a history of meningioma resection 18 years prior who presented 16 years later with an intractable ulceration around her left nasolabial sulcus. Pain and light‐touch sensations around the ulcer were decreased. She admitted to frequent manipulation due to a crawling sensation. A skin biopsy showed acanthotic changes and a decreased number of peripheral nerve fibers. Trigeminal trophic syndrome was diagnosed. Carbamazepine was not effective, and the ulcer persisted at 7 months after the initial presentation. We reviewed 36 English‐language publications from 2003 to 2012, and analyzed 61 cases of trigeminal trophic syndrome, including this patient. The mean age was 53.3 ± 19.7 years (range, 6–91). The right side of the face was more commonly affected (57%) than the left side. The ala nasi were involved in 48 cases (79%), followed by the cheek in 17 cases (28%). A corneal lesion was observed in 11 cases (18%), suggesting the importance of ophthalmologic consultations. The two major etiologies were trigeminal nerve ablation (18 cases; 30%) and cerebrovascular accidents (18 cases; 30%). The latent period ranged from days to 30 years. Gabapentin and carbamazepine were frequently administrated with variable efficacy. Application of thermoplastic dressings or negative pressure wound therapy demonstrated favorable outcomes. Surgery was an option with a high recurrence rate. Trigeminal trophic syndrome remains a clinical challenge.     

Trigeminal trophic syndrome in a pediatric patient with diffuse intrinsic pontine glioma

A 13-year-old girl with a history of diffuse intrinsic pontine glioma (DIPG) suffered from progressively worsening facial ulcerations secondary to paresthesia-induced self-excoriation. She was diagnosed with trigeminal trophic syndrome (TTS) induced by DIPG and struggled to heal her lesions in the background of this excoriation disorder. A multidisciplinary approach that included mood disorder management with sertraline and amitriptyline helped diminish paresthesia, improve her quality of life, and promote healing of the ulcers despite the progression of her DIPG. This case highlights the multifactorial complexity of TTS in pediatric patients and the need for successful management strategies.     

Trigeminal trophic syndrome

A case report of a 51-year-old woman who developed trigeminal trophic syndrome Following craniectomy is presented. This syndrome represents trophic ulceration by self-induced trauma to skin in the trigeminal area. Treatment of the present case was complicated by the patient's underlying psychological problems.     

Aphthous ulcers

Aphthous ulcers are one of the most common oral diseases worldwide. Their clinical presentation is characterized by multiple, recurrent, small, round, or ovoid ulcers with circumscribed margins and erythematous haloes present in different sizes. Oral lesions similar to aphthous ulcers may be present in several systemic diseases. This article will summarize the differential diagnosis of aphthous ulceration, with emphasis on a practical guide for the management of recurrent aphthous ulceration, including topical and systemic therapy.     

An unusual case of pemphigus vulgaris presenting as bilateral foot ulcers

We describe an unusual presentation of pemphigus vulgaris, an autoimmune intraepidermal blistering skin disease associated with autoantibodies to the desmosome glycoprotein, desmoglein 3. A 60-year-old man presented with bilateral ulceration on the dorsum of the feet. These clinical features persisted for 4 months before more characteristic signs of pemphigus vulgaris, including mouth ulceration and skin erosions, developed. The atypical presentation led to a delay in diagnosis and initiation of the appropriate treatment. Pemphigus vulgaris may have unusual manifestations, such as nail dystrophy, paronychia, or granulation tissue-like lesions, but this case of bilateral foot ulceration highlights a further, perhaps unique, clinical presentation of this autoimmune disease.     

Carbamazepine hypersensitivity syndrome mimicking mycosis fungoides

We report a case of a patient who experienced a generalized skin rash with systemic involvement 2 months after beginning carbamazepine treatment for trigeminal neuralgia. Skin biopsy specimens suggest mycosis fungoides. Complete remission of the clinical and pathologic changes after drug discontinuation and the positive result of carbamazepine from an epicutaneous test suggest a diagnosis of pseudolymphoma due to carbamazepine.     

Successful use of a thermoplastic dressing in two cases of the trigeminal trophic syndrome

Treatment of trigeminal trophic syndrome is challenging and often unsatisfactory, particularly in patients with cognitive impairment. We report the novel use of a thermoplastic dressing in two patients with trigeminal trophic syndrome. Use of the dressing resulted in successful healing of ulceration in both patients, which has been maintained in the short term, representing a simple and economical therapeutic option in the management of this difficult condition.     

Trigeminal trophic syndrome complicating a case of borderline tuberculoid leprosy

An example of trigeminal trophic syndrome presenting as ulceration of ala nasi in a case of borderline tuberculoid leprosy is reported. To the best of our knowledge, this is only the second case report of this manifestation in leprosy to be documented.