Description

A 56-year-old diabetic and hypertensive lady presented to us with a history of exertional precordial pain. On physical examination, we came across a very interesting finding of left-sided poliosis (whitening of eyebrow and eyelashes) with a patch of vitiligo over the left eyebrow (figure 1). In addition, there was a history of gradually decreasing visual acuity of the left eye and ipsilateral hypoacusis. A large hyperpigmented macule suggestive of a café-u-lait spot was noticed on the ipsilateral side of the neck. According to the history, the skin manifestations were present since 15 years of age and were non-progressive. There was no history of any epilepsy or mental retardation. CT head was essentially normal. Funduscopy was suggestive of mild retinal pigmentary changes in the left eye and no evidence of diabetic retinopathy in the other eye. Audiometry indicated mild ipsilateral sensorineural deafness. This constellation of findings (unilateral skin manifestations, fundus appearance and hypoacusis) befitted the diagnosis of Alezzandrini syndrome. Alezzandrini syndrome is a very rare and distinct clinical syndrome of unknown aetiology first described by Alezzandrini and Casala in 1959.1 Since the first observation, only few cases have been reported worldwide.2,–,5 This syndrome is characterised by unilateral tapetoretinal (retinal pigment epithelia) degeneration with the ipsilateral appearance of facial vitiligo and poliosis.6 A close differential is Vogt-Koyanagi-Harada syndrome characterised by bilateral decolouration of the skin, eyebrow, eyelashes, alopecia, chronic uveitis and meningoencephalitis.7 8 Our case is unique because here we have demonstrated ipsilateral café-u-lait spot on the neck in combination with the above findings. We could not trace any similar presentation in the medical literature.

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Figure 1

Photograph showing left-sided poliosis and café-u-lait patch.