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Alezzandrini syndrome
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Alezzandrini syndrome

Contributors: Mehdi Rashighi MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Alezzandrini syndrome is a very rare disorder that consists of unilateral depigmentation of the facial skin and hair associated with ipsilateral tapetoretinal (pigmentary retinal) degeneration and hearing loss. Only a handful of cases have been reported in the literature.

The cause of this syndrome is unknown, but one hypothesis is an autoimmune disease targeting melanocytes and other neural crest derivatives. The clinical features of Alezzandrini syndrome overlap with Vogt-Koyanagi-Harada (VKH) syndrome, although Alezzandrini syndrome symptoms characteristically present unilaterally, leading some experts to believe they are manifestations of the same underlying disease process.

Patients present with unilateral depigmentation of the facial hair and skin and ipsilateral loss of visual and auditory acuity. The syndrome progresses over years.

Codes

ICD10CM:
L81.9 – Disorder of pigmentation, unspecified

SNOMEDCT:
403275004 – Alezzandrini syndrome

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Segmental vitiligo – Typically not associated with visual or auditory signs or symptoms.
  • VKH syndrome – Vitiligo, poliosis, and ocular and auditory findings are typically bilateral.
  • Piebaldism – Poliosis of forelock is typically associated with widespread, symmetrical depigmentation of central face, trunk, and extremities. No associated ocular or auditory abnormalities.

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed:03/02/2017
Last Updated:03/29/2017
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Alezzandrini syndrome
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Alezzandrini syndrome : Hearing loss, Vision loss, White patch
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