|Year : 2015 | Volume
| Issue : 1 | Page : 62-64
Is it Vogt-Koyanagi-Harada syndrome?
Digambar Dashatwar, Neha Singh, Sumit Kar
Department of Dermatology, Venereology and Leprosy, Mahatma Gandhi Institute of Medical Sciences, Wardha, Maharashtra, India
|Date of Web Publication||8-Jan-2015|
Department of Dermatology, Venereology and Leprosy, Mahatma Gandhi Institute of Medical Sciences, Sewagram, Wardha - 442 102, Maharashtra
Source of Support: None, Conflict of Interest: None
Vogt-Koyanagi-Harada syndrome (VKH syndrome) is named after ophthalmologists Alfred Vogt from Switzerland and Yoshizo Koyanagi and Einosuke Harada from Japan. It is an uncommon multisystem disorder characterized by chronic, bilateral, diffuse granulomatous uveitis with associated dermatological, neurological and auditory manifestations. It is an autoimmune disease directed against melanocytes. We report a case presenting with vitiligo, auditory defect with additional dextrocardia.
Keywords: VKH syndrome, dextrocardia, spina bifida of D2 vertebra
|How to cite this article:|
Dashatwar D, Singh N, Kar S. Is it Vogt-Koyanagi-Harada syndrome?. Med J DY Patil Univ 2015;8:62-4
| Introduction|| |
VKH Syndrome presents itself in 4 stages; the prodromal phase, the acute uveitic phase, the convalescent phase and lastly the chronic recurrent phase. The prodromal stage is characterized by flu-like symptoms, headache, nausea, meningism, dysacusia (discomfort caused by loud noises) and tinnitus. The acute uveitic stage is heralded by the onset of bilateral blurring of vision, bilateral granulomatous anterior uveitis, vitritis, thickening of the posterior choroid, hyperemia and edema of the optic nerve and retinal detachment. The convalescent stage occurs several weeks later and is characterized by depigmentation of the choroid, seen as a sunset glow appearance on fundoscopy. Skin changes begin to appear during this stage and include vitiligo, alopecia and poliosis. The chronic recurrent stage is marked by repeated bouts of uveitis.
| Case Report|| |
An 11-year-old boy presented to us with vitiligo vulgaris [Figure 1]. He had severe right sided hearing loss. He had no ocular complains. There was no history of parental consanguity. His mother suffered from congenital bilateral sensorineural deafness. His younger brother was also diagnosed to have vitiligo. His chest radiograph revealed dextrocardia [Figure 2] with abdominal situs in-situ. A large diaphragmatic hernia was evident on right side with herniation of liver into the thorax. Ultrasonography of abdomen showed poor movement of the right leaflet of diaphragm on respiratory movement. Echocardiogram and color doppler study showed isolated dextrocardia. His X-ray of spine revealed spina bifida of first thorasic vertebra. Audiometry done 5 years back showed profound sensorineural hearing loss of right ear [Figure 3]. This patient had vitiligo with leucotrichia of scalp hair, unilateral sensorineural hearing loss and episodes of life threatening neurological complications during infancy. At the age of 6 months child had a severe episode of acute febrile illness with febrile convulsions suggestive of meningoencephilits. For treating it patient was hospitalized in ICU in a hospital. Clinical notes available with patient do not show details of lumbar puncture during hospitalization for severe life threatening illness in infancy. Thus, a diagnosis of VKH syndrome was made.
Corticosteroids are the mainstay of treatment in this syndrome. For repigmentation of depigmented macules topical potent corticosteroid ointment and topical calcineurin inhibitor ointment are being tried with satisfactory repigmentation of depigmented macules. Oral pulsed corticosteroids have been given to control appearance of new depigmented macules and spreading of existing depigmenting lesions.
| Discussion|| |
Dextrocardia and spina bifida of dorsal vertebra have not been reported to be associated with Vogt Koyanagi Harada (VKH) syndrome in spite of extensive search of world literature. The purpose of this communication is to report here the unreported association of dextrocardia and spina bifida in a patient of VKH syndrome. VKH syndrome is an immune-mediated disease, wherein the underlying pathophysiology is T helper cell mediated autoimmune attack on melanocytes of the skin, uvea, central nervous system and the inner ear. VKH syndrome occurs more commonly in patients with a genetic predisposition  to the disease, so does our patient have. VKH syndrome may be associated with other autoimmune disorders such as hypothyroidism, Hashimoto thyroiditis, diabetes mellitus,  Guillain-Barre syndrome  and IgA nephropathy.  This syndrome has also been reported to be linked to malignant lymphoma.  Conditions that need to be differentiated from Vogt-Koyanagi-Harada syndrome include tuberculosis, alopecia areata, piebaldism, sarcoidosis, syphilis and intraocular lymphoma.
| Conclusion|| |
VKH syndrome in children often goes unrecognized.  Though the disease is not associated with mortality, the long-term complications of VKH syndrome include vision loss due to cataract, glaucoma and choroidal neovascularization. Our patient had depigmented macules over face, trunk and limbs, leukotrichia of scalp hair. He suffered from profound sensorineural hearing loss in both ears 5 years ago and needs hearing aids. Early diagnosis and prompt treatment may prevent devastating complications and help in optimal development of child.
Our patient had dextrocardia and spina bifida, heretofore unreported associations in VKH syndrome. The patient is being followed up.
| References|| |
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[Figure 1], [Figure 2], [Figure 3]