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Alezzandrini Syndrome Clinical Presentation

  • Author: Camila K Janniger, MD; Chief Editor: William D James, MD  more...
 
Updated: Jun 21, 2016
 

History

A gradual loss of visual acuity initially occurs, predominantly in one eye. The hair and skin in affected areas lose their pigmentation. In one patient, ipsilateral hypoacusis was a late finding. Bilateral deafness developed in one patient. This disorder may be first evident with unilateral vitiligo on the cheek and partial loss of hearing and vision on the same side.[15]

Alopecia areata is a common disorder that has been described in association with Vogt-Koyanagi-Harada syndrome.[16] One should delineate the alopecia and poliosis linked with Vogt-Koyanagi-Harada syndrome from alopecia areata. Vogt-Koyanagi-Harada disease may be first evident as a headache.[17, 18]

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Physical

Alezzandrini syndrome is characterized by unilateral tapetoretinal (retinal pigmented epithelia) degeneration with the ipsilateral appearance of facial vitiligo and poliosis, as shown in the image below.

Gray forelock and vitiligo on the forehead in a pa Gray forelock and vitiligo on the forehead in a patient with Alezzandrini syndrome.

Ipsilateral perceptual deafness may be an additional finding. Unilateral cutaneous and retinal disease also characterize Alezzandrini syndrome. The symptom originally described was a gradual loss of visual acuity, which predominantly affected one eye. Lorincz[19] believes that the gradual loss of visual acuity on the ipsilateral side is the criterion that should be used to diagnosis Alezzandrini syndrome.

After 3-13 years, vitiligo and poliosis develop on the head, on the side ipsilateral to the retinal lesions. Two most highly specific, at least in those with Vogt-Koyanagi-Harada disease, are exudative retinal detachment during acute disease and sunset glow fundus during the chronic phase of the disease.[20]

Congenital leukoderma suggests the need for evaluation of ocular, auditory, and/or neurologic abnormalities.[21] Poliosis may be acquired in vitiligo, Vogt-Koyanagi-Harada syndrome, Alezzandrini syndrome, and sarcoidosis. However, vitiligo patients have more lenticular and retinal changes than one should anticipate.[22]

A patient was described with a large hyperpigmented macule suggestive of an ipsilateral café-au-lait spot on the neck.[23]

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Causes

The condition has an unknown etiology. Several theories, including those involving viral or autoimmune processes, are postulated.

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Contributor Information and Disclosures
Author

Camila K Janniger, MD Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, Rutgers New Jersey Medical School

Camila K Janniger, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Ewa Koziorynska, MD Assistant Professor of Neurology, Comprehensive Epilepsy Center, State University of New York Downstate Medical Center

Ewa Koziorynska, MD is a member of the following medical societies: Sigma Xi

Disclosure: Received none from none for none.

Specialty Editor Board

Michael J Wells, MD, FAAD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Noah S Scheinfeld, JD, MD, FAAD Assistant Clinical Professor, Department of Dermatology, Weil Cornell Medical College; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Assistant Attending Dermatologist, New York Presbyterian Hospital; Assistant Attending Dermatologist, Lenox Hill Hospital, North Shore-LIJ Health System; Private Practice

Noah S Scheinfeld, JD, MD, FAAD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Abbvie<br/>Received income in an amount equal to or greater than $250 from: Optigenex<br/>Received salary from Optigenex for employment.

References
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  2. Alezzandrini AA. [Unilateral manifestations of tapeto-retinal degeneration, vitiligo, poliosis, grey hair and hypoacousia]. Ophthalmologica. 1964. 147:409-19. [Medline].

  3. Barnes L. Vitiligo and the Vogt-Koyanagi-Harada syndrome. Dermatol Clin. 1988 Apr. 6(2):229-39. [Medline].

  4. Vogt A. Fruhzeities ergraven der zilien und bemerkungen uber den sogenannten plotzlichen einendieser veranderugn. Klin Monatsbl Augenheilkd. 1906. 44:228.

  5. Gilbert W. Vitiligo und Auge, ein Beitrag zur Kenntnis der herpetischen augeenerkran kunger. Klin Monatsbl Augenheilkd. 1910. 48:24.

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  17. A V P, Kumar J S A, K N N, Rao S, Shetty S. Unusual Case of Vogt-Koyanagi-Harada Syndrome Presenting as Non-specific Headache. J Clin Diagn Res. 2014 Apr. 8(4):VD06-VD07. [Medline]. [Full Text].

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Gray forelock and vitiligo on the forehead in a patient with Alezzandrini syndrome.
 
 
 
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