SHORT COMMUNICATION 971
ActaDV ActaDV
Advances in dermatology and venereology Acta Dermato-Venereologica
Atypical Trigeminal Trophic Syndrome : An Unusual Cause of Facial Ulceration
Jonathan KENTLEY 1 , Claire MARSHALL 2 , Maria-Angeliki GKINI 1 , Ruth TAYLOR 3 and Anthony BEWLEY 1 , 4 * Departments of 1 Dermatology and 3 Psychiatry , Royal London Hospital , Barts Health NHS Trust , London , 2 Department of Dermatology , York Hospital , York , and 4 Whipps Cross University Hospital , Whipps Cross Road , E11 1NR London , UK . * E-mail : Anthony . Bewley @ bartshealth . nhs . uk Accepted Apr 18 , 2017 ; Epub ahead of print Apr 19 , 2017
In patients presenting with facial ulceration , a number of causes should be considered : including malignancy , vasculitis , infection and psychodermatological disease . Trigeminal trophic syndrome ( TTS ) is a rare but important cause of facial ulceration resulting from damage to the trigeminal nerve , resulting in self-mutilating behaviour and ulceration . We present a case of atypical trigeminal trophic syndrome ( ATTS ) manifesting with severe , bilateral disease highly refractory to conventional therapy . We stress the importance of recognising this condition and involving a multidisciplinary team in management of these challenging patients .
CASE REPORT
A 56-year-old man presented to our service a 6-year history of chronic facial pain , ulceration and subsequent scarring ; which was preceded by an episode of a condition diagnosed in primary care as shingles . The left and right cheeks , left post-auricular area and chin were affected ( Fig . 1 ). These changes had previously been diagnosed as secondary to dermatitis artefacta or acne excoriée . In the past he had used antidepressant medications and interacted with a psychologist , but terminated these therapies due to lack of perceived response .
Differential diagnoses of facial ulceration including the following were considered : malignancy , infection , vasculitis , pyoderma gangrenosum and psychodermatological diseases : dermatitis artefacta ( DA ), TTS and acne excoriée .
Swabs from active ulcers grew normal skin flora . Bloods revealed a microcytic anaemia , with no evidence
Fig . 1 . Bilateral ulceration and scarring affecting both cheeks , left postauricular region and chin . of gastrointestinal bleeding found . Additional biochemical tests for pruritus were normal ( liver function , urea and electrolytes , thyroid function , folate and Treponema serology ). Skin biopsy noted only scarring of the epidermis . Magnetic resonance cranio-facial imaging showed mild chronic inflammation of the facial sinuses . Neurophysiology revealed bilateral trigeminal nerve dysfunction on both trigeminal nerve somatosensory evoked potentials and electromyography .
Our patient has been treated with simple analgesia ( paracetamol and codeine ); an antihistamine : hydroxyzine ; selective serotonin re-uptake inhibitors : escitalopram , fluoxetine , paroxetine , sertraline ; a tricyclic antidepressant : amitriptyline ; antiepileptic drugs : gabapentin , pregabalin , topiramate and antipsychotics : quetiapine , olanzapine and amisulpride . All of these therapies achieved limited success . After lengthy consultation with pain services our patient is currently maintained on aripiprazole ( 5 mg daily ), morphine sulphate ( 40 mg twice daily ( BD )), pregabalin ( 300 mg BD ), duloxetine ( 60 mg BD ), oxcarbazepine 150 mg BD and topical lidocaine patches .
DISCUSSION
TTS is a rare but important cause of facial ulceration and consists of a triad of ulceration , anaesthesia and paraesthesia . TTS results from injury to the trigeminal nerve , which may be central or peripheral , and the resulting intractable dysasthesia leads to self-mutilating behaviour resulting in ulcers ( 1 ). These chronic ulcerating lesions can typically be observed in the nasal ala and paranasal areas but may be seen to involve any of the ophthalmic ( V 1
) maxillary ( V 2 ) or mandibular ( V 3
) nerve distributions ( 1 , 2 ). Ulceration is characteristically unilateral and may occur as single or multiple lesions ( 3 , 4 ).
The diagnosis is often made clinically . Patients are likely to report a preceding condition accounting for insult to the trigeminal nerve ; namely stroke , trigeminal neuralgia , herpes zoster , meningioma , acoustic neuroma , encephalitis , syphilis or surgical procedures affecting the nerve ( 1 ). Patients are commonly misdiagnosed with DA , but the intractable facial sensations described by the patient and characteristic distribution should suggest a diagnosis of TTS ( 5 ). Histology is often non-specific . Neurophysiological studies can be helpful in evaluating function of the trigeminal nerve . Often a multi-disciplinary team ( dermatologists , neurologists , psychiatrist and occasionally surgeons ) is required to
This is an open access article under the CC BY-NC license . www . medicaljournals . se / acta Journal Compilation © 2017 Acta Dermato-Venereologica . doi : 10.2340 / 00015555-2675 Acta Derm Venereol 2017 ; 97 : 971 – 972