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Alezzandrini syndrome

Author: Dr Vanessa Van de Velde, Registrar; Dr Alex Holme, Dermatologist, Royal Infirmary of Edinburgh, Edinburgh, Scotland; Chief Editor: A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand, March 2016.

Alezzandrini syndrome — codes and concepts

What is Alezzandrini syndrome?

Alezzandrini syndrome is characterised by depigmentation.

  • Unilateral degeneration of the pigmented layer of the retina
  • Ipsilateral facial vitiligo
  • Poliosis (loss of colour from a patch of hair)
  • Reduced hearing 

Alezzandrini syndrome has been closely associated with Vogt-Koyanagi-Harada (VKH) syndrome, as they share similar cutaneous and ocular signs. Neurological involvement typical of VKH syndrome (8th cranial nerve damage and sterile meningitis) are not reported in Alezzandrini syndrome.  The connection between the syndromes remains unknown.

Who gets Alezzandrini syndrome?

Only a handful of patients with Alezzandrini syndrome have been described, who were males and females between 12 and 30 years old at presentation.

What causes Alezzandrini syndrome?

The cause of Alezzandrini syndrome remains unknown. Viral or autoimmune processes leading to melanocyte destruction have been suggested. Melanocytes migrate to the skin, retinas, uvea, cochleae, and vestibular labyrinths so any of these sites may be affected.

What are the clinical features of Alezzandrini syndrome?

The initial presentation may be unilateral gradual loss of vision, followed by ipsilateral facial vitiligo and poliosis several years later. Ipsilateral reduced sensitivity to sound or hypoacusis may also develop. Bilateral deafness has been reported in one patient.

Loss of pigmentation of skin and hair

How is Alezzandrini syndrome diagnosed?

Alezzandrini syndrome is diagnosed on recognising the clinical presentation.

Ophthalmological investigations to assess for complications may include regular:

  • Fundus examination
  • Visual acuity testing
  • Fundus autofluorescence
  • Spectral domain-optical coherence tomography imaging 

Audiometry is undertaken to assess hearing loss.

What is the treatment for Alezzandrini syndrome?

There are no treatments for Alezzandrini syndrome.

What is the outcome for Alezzandrini syndrome?

Due to the small number of reported cases, the prognosis of patients with this syndrome remains unknown.

Patients experience a gradual loss of vision and in some cases, progressive hearing loss. They have a greater risk of retinal detachment.

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