Alezzandrini's Disease Simulating the Vogt-Koyanagi-Harada Syndrome

S. Shamsadini MD,* M.R. Meshkat MD,** K. Mozzafarinia MD***

Department of *Dermatology, **Ophthalmology, and ***ENT, Kerman Darman Hospital, Kerman University of Medical Sciences, Kerman, Iran

Introduction

The association of vitiligo and inflammatory eye disease has been recognized for several decades as the Vogt-Koyanagi-Harada (VKH) syndrome.¹ The syndrome is characterized by association of generalized vitiligo, poliosis, chronic uveitis, dysacousia, alopecia and meningitic symptoms. This syndrome represents a combination of two separately described disorders: the Vogt-Koyanagi syndrome and Harada's disease. Another related disease described by Alezzandrini in 1964 is characterized by unilateral facial vitiligo and partial poliosis with ipsilateral tapetoretinal degeneration that may be combined with hypoacusis.^2,3 This rare syndrome of unknown origin has been reported in adolescents and young adults. Unilateral impairment of vision takes place from a degenerative retinitis followed, within an interval of months or years, by facial vitiligo and poliosis on the same side. Bilateral perceptive deafness may also develop.^4,5,6 Herein we report a case of AS that mimics some features of VKH syndrome.

Case Report

A 38-year-old blind man was referred to the Dermatology Clinic of the Kerman Hospital presenting with vitiligo of the infraorbital area on the right side, and poliosis of the eyebrow and eyelashes. The disease began approximately 22 years ago, when he noticed that he had trouble with his vision, especially on his right side. Ophthalmologic examination showed retinal detachment and because of severe retinal degeneration, surgical operation for retinal reattachment was not successful. Ipsilateral poliosis of the forelock had appeared 1 year later. After three years, he felt that something was wrong with his left eye; a similar experience he had gone through with his right eye and finally, he became completely blind. One year later, his wife noticed an increasingly large whitish infraorbital patch (1.5 ´ 3 cm with a regular sharp margin) on the right side (Fig. 1). Thus, a diagnosis of AS with poliosis of the forelock on the right side associated with ipsilateral eyebrow and eyelashes was made (Fig. 2).

During the past 6 months the patient reported increasing trouble with his right ear. Audiometric examination showed moderate neurological sensory deafness at the frequencies of more than 3000 Hz. He also complained of hypoacusis which was worse than what would be anticipated from his audiometric findings and might have been due to other factors such as abnormality in the internal ear stria vascularization.

His past medical history was unremarkable except for two admissions to a general hospital for a disk herniation at L3-L4 nine years ago and another herniated disc at L1-L2 which occurred one year ago. The patient's family history revealed that his grandfather had become blind at the age of 19, but the cause of his blindness was unknown. His only brother also has acral asymmetric vitiligo, without any ophthalmological disorder or poliosis.

Discussion

Retinal detachment is seen in many pigmentary disorders, such as Vogt-Koyanagi-Harada (VKH) and Alezzandrini's syndrome(AS). VKH is a triphasic disease. The first stage or prodrome is dominated by signs of meningeal irritation. The second phase constitutes ophthalmic and auditory involvement leading to gradual loss of vision.¹ No history of meningeal involvement was elicited in this case, therefore, the diagnosis of AS or Vogt-Koyanagi (VK) but not VKH was our first consideration. Retinal detachment is seen more common in AS and only in VKH syndrome, but not in the VK syndrome alone^2,4.

Although retinal detachment is usually seen unilaterally in AS, bilateral retinal detachment was noted in our patient although not synchronously (first on the right and then on the left side). Our observations are in keeping with those of Lorincz's. The feature that distinguishes Alezzandrini's syndrome is a unilateral vitiliginising pigmentary disorder that involves the skin, retina and in some cases acoustic nerve.^4,6

The initial symptom in patients with Alezzandrini's syndrome is a gradual loss of vision, predominantly in one eye.² Lornicz believes that the criteria used to determine the diagnosis of AS

might need to be broadened to include retinal detach-ment.^7,8 In the present case retinal detachment appeared as a prodromal sign and occurred bilaterally. Therefore, one can consider that Alezzandrini's syndrome is a variety of melanocytic degenerations including vitiligo and VKH syndrome.⁶

References

1 Arnold HL, Odom RB, James WD. Andrew's Diseases of the Skin. 8th ed. Philadelphia: Saunders Co,1990.

2 Mark D, Hoffman MD, Dudley C (MD Chicago, Illinois). Suspected Alezzandrini's syndrome in a diabetic patient with unilateral retinal detachment. Am Acad Dermatol 1992;26:496-7.

3 Ortonne JP, Mosher DB, Fitzpatrick TB. Miscellaneous hypomelanosis. In: Ortonne JP, et al (eds): Vitiligo and Other Hypomelanosis of Hair and Skin. New York: Plenum Medical Book Co, 1983.

4 Alezzandrini AA. Manifestations unilaterales de dégénerence tapéto-retinienne, de vitiligo, de poliose de cheveux blancs et d'hypoacousie. Ophthalmologica 1964;174:409-19.

5 Carrasquillo HR. Uveitis with poliosis, vitiligo, alopecia and dysacousia(Vogt Koyanagi not Harada syndrome). Arch Ophthalmol 1942;28:385-414.

6 Cremona AC, Alezzandrini AA, Casala AM. Vitiligo degeneration macular unilateral. Arch Opthal B Aires 1961;36:102-6.

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

8 Casala AM, Alezzandrini AA. Vitiligo, poliosis, unilateral conretinities pigmentaria hypoacusia Arch Argent Derm 1959;9:449-56.

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