BSD 2015 Case 17

Male 77. Ulcer left side of nose biopsied. Past history of surgery for trigeminal neuralgia.The best diagnosis is:

A. ulcer secondary to herpes virus infectionB. ulcer of pyoderma gangrenosumC. ulcerated microvenular haemangiomaD. ulcer of trigeminal trophic syndrome

BSD 2015 Case 17

Further biopsy 2 years later

Another further biopsy also 2 years later

BSD 2015 Case 17

Male 77. Ulcer left side of nose biopsied. Past history of surgery for trigeminal neuralgia. The best diagnosis is:

A. ulcer secondary to herpes virus infectionB. ulcer of pyoderma gangrenosumC. ulcerated microvenular haemangiomaD. ulcer of trigeminal trophic syndrome

BSD 2015 Case 17

Male 77. Ulcer left side of nose biopsied. Past history of surgery for trigeminal neuralgia. The best diagnosis is:

A. ulcer secondary to herpes virus infectionB. ulcer of pyoderma gangrenosumC. ulcerated microvenular haemangiomaD. ulcer of trigeminal trophic syndrome

Trigeminal trophic syndrome(trigeminal neurotrophic ulceration/

trophic ulceration of the ala nasi)

• Damage to any branch of trigeminal nerve

• Mainly ala nasi (79%) and cheek (28%)

• also lip, forehead, scalp, ear, jaw, palate

Trigeminal trophic syndrome

• Commonest causes:

– surgical treatment of trigeminal neuralgia (30%)

– stroke/cerebrovascular accident (30%) - posterior inferior cerebellar artery or vestibular artery dissection

– surgical treatment: complication of neurosurgery

– other trauma

– herpes zoster (or simplex)

– leprosy, syphilis, post-encephalitis or birth trauma etc.

Trigeminal trophic syndrome;clinical features

• mean 53 years (range 6-91)

• female in 69%

• A few weeks up to 30 years after nerve damage

• Ulceration, anaesthesia and paraesthesia

• Unilateral ulcer, but may be multiple

• Cornea involved in 18%,

mostly where corneal reflex lost

Sawada T, Asai J, Nomiyama T, Masuda K, Takenaka H, Katoh N. Trigeminal trophic syndrome: report of a case and review of the published work. J Dermatol. 2014 Jun;41(6):525-8

Trigeminal trophic syndrome;histological features

• Non-specific

• Deep ulceration –

look for unusually superficial muscle, large blood vessels etc.

• Pseudoepitheliomatous hyperplasia

• Scarring

• Vascular proliferation

• No herpes or malignancy!

Trigeminal trophic syndrome;treatment

• Very difficult – cannot treat anaesthesia so the scratching/trauma continues

• paraesthesia and ulceration treatment– pharmacological (antineuropathic agents such as gabapentin,

carbamazepine)

– Transcutaneous electrical nerve stimulation (TENS)

– Surgical reconstruction with innervated skin flaps – but high recurrence

– Sympathectomy

– thermoplastic dressings or negative pressure wound therapy

– Reduce trauma – cut finger nails, nocturnal scratch mittens

Carbamazepine as the only effective treatment in a 52-year-old man with trigeminal trophic syndrome. Fruhauf J, Schaider H, Massone C, Kerl H, Mullegger RR. Mayo Clin Proc. 2008 Apr;83(4):502-4

The “immunocompromised cutaneous district”

• A complex dysregulation of neuropeptides is a feature of some diseases of both dermatological and neurological interest

Dermatological and immunological conditions due to nerve lesions. Bove D, Lupoli A, Caccavale S, Piccolo V, Ruocco E. Funct Neurol. 2013 Apr-May;28(2):83-91.

Neurologic causes of the “immunocompromised cutaneous district”

– Infections:

• (PNS) herpes zoster and simplex

• (CNS) poliomyelitis

– Ischaemia

• Cerbral stroke (cardiovascular accident)

• Cerebellar stroke)

– Nerve compression: carpal tunnel syndrome

– Nerve palsy: facial nerve palsy

– Other/multiple causes : trigeminal trophic syndrome