Article Text

Unilateral facial ulceration and Horner's syndrome

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A 55-year-old diabetic and hypertensive woman presented acutely with vertigo and vomiting. She had right-sided ataxia, horizontal right gaze-evoked nystagmus, and a right miosis. Later, a right ptosis was noted. Temperature and pinprick sensations on the right side of the face and left side of the body were disturbed. Examination of the precordium was normal. Computed tomography of the head showed moderate generalised cerebral atrophy and an old left occipital infarct. There was no evidence of brainstem or cerebellar haemorrhage.

Six months later the patient presented with excoriation and ulceration affecting the right eyelid, temple and frontal scalp. She complained of persistent itch in these sites. Swabs from the ulcerated areas were negative, including on viral culture. She was prescribed topical 2% fucidic acid / 0.1% betamethasone valerate cream and 1% ichthammol in zinc ointment, and advised to avoid scratching. Four years later, however, the same lesions are still noted, as well as ulceration affecting the right lower lip and a healing ulcer on the right nasal ala (figure). The right-sided Horner's syndrome and crossed sensory loss persist, but her ataxia and nystagmus have resolved.

Figure The face of the patient (reproduced with her permission)

Questions

1
What was the initial diagnosis?
2
What is the differential diagnosis of the nasal ulceration?
3
What caused this patient's facial ulceration?

Answers

QUESTION 1

The patient had a right-sided lateral medullary infarction or Wallenberg's syndrome following occlusion of the right vertebral or posterior inferior cerebellar artery.

QUESTION 2

The differential diagnosis of nasal ulceration involves infective, neoplastic and autoimmune aetiologies (box FB1), dermatitis artefacta and trigeminal trophic syndrome.

Figure FB1

QUESTION 3

Trigeminal trophic syndrome. This is a recognised complication of lateral medullary infarction (box FB2).

Figure FB2
Figure FB3

Discussion

Neurotrophic ulceration in the trigeminal nerve dermatome, usually the maxillary division, was first recognised independently by Lovemann1 and McKenzie2 in 1933. Most reported cases of the trigeminal trophic syndrome (TTS), as it is now termed, have followed treatments of trigeminal neuralgia by trigeminal rhizotomy or alcohol injection into the Gasserian ganglion. Other recognised causes include acoustic neuroma, postencephalitic Parkinsonism, syringobulbia and vertebrobasilar insufficiency (box FB2).

In TTS there is evidence of dermatomally defined (self-inflicted) trauma on the face. Most published cases describe involvement of the nasal ala. It is thought to arise from impairment of the protective sensory feedback mechanism in the setting of an irritative focus. Even following trigeminal rhizotomy or alcohol injection into the Gasserian ganglion, the syndrome only occurs in a small proportion of patients. There are fewer than 20 previously published cases of TTS complicating Wallenberg's syndrome, all involving the nasal ala.3-5Lesions in the distribution of Va were also described in some of these patients. In our patient trophic ulceration occurred in all three divisions of the trigeminal nerve secondary to Wallenberg's syndrome.

Despite the clinical uniformity, the brainstem lesions accompanying Wallenberg's syndrome are heterogenous. Neurophysiological abnormalities are limited to the afferent arm of the reflex circuit.6 Fibres from the ophthalmic division of the trigeminal nerve reach the lowermost part of the (trigeminal) spinal nuclei, and fibres from the maxillary and mandibular divisions lie more rostrally.6 The current patient's sensory disturbance reflects extensive trigeminal tract involvement. In addition to impaired somatic sensation, faulty sympathetic innervation of cutaneous blood vessels may also contribute to the persistent ulceration. The Horner's syndrome in our patient confirms residual sympathetic damage.

TTS following Wallenberg's syndrome can develop from a fortnight to many months after the stroke, and may arise at different times within the same nerve division. Management involves prevention of further trauma and antibiotics to control secondary infection. Transposition of an innervated skin flap from the unaffected side and transcutaneous electrical stimulation7 have also been suggested in anecdotal reports. Our patient continues on conservative treatment.

Final diagnosis

Trigeminal trophic syndrome following Wallenberg's syndrome.

References

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