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.
L81.9 – Disorder of pigmentation, unspecified
403275004 – Alezzandrini syndrome
Differential Diagnosis & Pitfalls
- – Typically not associated with visual or auditory signs or symptoms.
- – Vitiligo, poliosis, and ocular and auditory findings are typically bilateral.
- – Poliosis of forelock is typically associated with widespread, symmetrical depigmentation of central face, trunk, and extremities. No associated ocular or auditory abnormalities.