Alezzandrini Syndrome Clinical Presentation

  • Author: Camila K Janniger, MD; Chief Editor: William D James, MD   more...
 
Updated: May 27, 2011
 

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.[12]

Alopecia areata is a common disorder that has been described in association with Vogt-Koyanagi-Harada syndrome.[13] One should delineate the alopecia and poliosis linked with Vogt-Koyanagi-Harada syndrome from alopecia areata.

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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 paGray 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[14] 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.[15]

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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, University of Medicine and Dentistry of New Jersey-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: none None None

Specialty Editor Board

Noah S Scheinfeld, MD, JD, FAAD  Assistant Clinical Professor, Department of Dermatology, Columbia University College of Physicians and Surgeons; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, and New York Eye and Ear Infirmary; Private Practice

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

Disclosure: Optigenex Consulting fee Independent contractor

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

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

Disclosure: Nothing to disclose.

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

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

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD  Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System

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

Disclosure: Elsevier Royalty Other

References

  1. Casala AM, Alezzandrini AA. Vitiligo, poliosis unilateral con retinitis pigmentaria y hypoacusia. Arch Argent Dermatol. 1959;9:449.

  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. Apr 1988;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.

  6. Harada E. Clinical study of nonsuppurative choroiditis: A report of acute diffuse choroiditis. Acta Soc Opthalmol Jpn. 1926;30:356.

  7. Koyanagi Y. Dysakusis, Alopecia, und Poliosis beschwerer Uveitis nicht traumatischen Ursprunge. Klin Monatsbl Augenheilkd. 1929;82:194.

  8. Kodjikian L, Seve P, Le Hoang P, Garweg JG. Atypical Vogt-Koyanagi-Harada disease or new uveomeningitic syndrome?. Graefes Arch Clin Exp Ophthalmol. Mar 2005;243(3):263-5. [Medline].

  9. Du L, Yang P, Hou S, Lin X, Zhou H, Huang X, et al. Association of the CTLA-4 gene with Vogt-Koyanagi-Harada syndrome. Clin Immunol. Apr 2008;127(1):43-8. [Medline].

  10. Hoffman MD, Dudley C. Suspected Alezzandrini's syndrome in a diabetic patient with unilateral retinal detachment and ipsilateral vitiligo and poliosis. J Am Acad Dermatol. Mar 1992;26(3 Pt 2):496-7. [Medline].

  11. Shamsadini S, Meshkat MR, Mozzafarinia K. Bilateral retinal detachment in Alezzandrini's syndrome. Int J Dermatol. Dec 1994;33(12):885-6. [Medline].

  12. Andrade A, Pithon M. Alezzandrini syndrome: report of a sixth clinical case. Dermatology. Feb 2011;222(1):8-9. [Medline].

  13. Haque WM, Mir MR, Hsu S. Vogt-Koyanagi-Harada syndrome: Association with alopecia areata. Dermatol Online J. 2009;15(12):10. [Medline].

  14. Lorincz AL. Disturbances of melanin pigmentation. In: Moschella SL, Hurley JH, eds. Dermatology. Philadelphia, Pa: WB Saunders; 1985.

  15. Rao NA, Gupta A, Dustin L, Chee SP, Okada AA, Khairallah M, et al. Frequency of distinguishing clinical features in Vogt-Koyanagi-Harada disease. Ophthalmology. Mar 2010;117(3):591-9, 599.e1. [Medline]. [Full Text].

  16. Huggins RH, Janusz CA, Schwartz RA. Vitiligo: a sign of systemic disease. Indian J Dermatol Venereol Leprol. Jan-Feb 2006;72(1):68-71. [Medline].

  17. Vasconcelos-Santos DV, Sohn EH, Sadda S, Rao NA. Retinal pigment epithelial changes in chronic Vogt-Koyanagi-Harada disease: fundus autofluorescence and spectral domain-optical coherence tomography findings. Retina. Jan 2010;30(1):33-41. [Medline].

  18. Zamecki KJ, Jabs DA. HLA typing in uveitis: use and misuse. Am J Ophthalmol. Feb 2010;149(2):189-193.e2. [Medline].

  19. Raffa L, Bawazeer A. Intravitreal bevacizumab injection in a 14-year-old Vogt-Koyanagi-Harada patient with choroidal neovascular membrane. Can J Ophthalmol. 2009;44:615-6. [Medline].

  20. Khalifa YM, Bailony MR, Acharya NR. Treatment of pediatric vogt-koyanagi-harada syndrome with infliximab. Ocul Immunol Inflamm. Jun 2010;18(3):218-22. [Medline].

  21. Chee SP, Jap A, Bacsal K. Prognostic factors of Vogt-Koyanagi-Harada disease in Singapore. Am J Ophthalmol. Jan 2009;147(1):154-161.e1. [Medline].

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