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.
Alezzandrini syndrome
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Synopsis
Codes
ICD10CM:
L81.9 – Disorder of pigmentation, unspecified
SNOMEDCT:
403275004 – Alezzandrini syndrome
L81.9 – Disorder of pigmentation, unspecified
SNOMEDCT:
403275004 – Alezzandrini syndrome
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Last Reviewed:03/02/2017
Last Updated:03/29/2017
Last Updated:03/29/2017
Alezzandrini syndrome