Abstract
Background Vogt Koyanagi Harada (VKH) is a syndrome most commonly presenting between the second and fifth decades of life.1,2 Its incidence in children is rare, with a much graver prognosis and worse visual outcome. Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy syndrome (APECED) is a rare genetic (autosomal recessive) disorder. It was first reported in 1929 under the name of type 1 autoimmune polyglandular syndrome (APS1) or Whitaker’s syndrome.3 It is characterized by the presence of two or more endocrine disorders with immunological pathogenesis, resulting in a hypofunctional state.
The clinical picture is variable, but chronic mucocutaneous candidiasis is typically the presenting symptom.4 Other autoimmune manifestations often ensue, most commonly Addison’s disease, hypothyroidism, and gonadal failure.
Insulin-dependent diabetes, alopecia areata, vitiligo, ectodermal dystrophy, nail dysplasia, and ocular symptoms (keratoconjunctivitis, iridocyclitis, cataract, and optic atrophy) are less common manifestations.
Case Report(s) A two-year-old type 1 diabetic with hypothyroidism presented with impaired fixation. Ocular examination revealed right vitritis, choroiditis, a hyperemic disc, and an area of exudative detachment. At the same time, there was no fundus view in the left eye, and ultrasonographic assessment revealed vitritis and a thickened choroid. Patient developed sunset glow fundus with alopecia, poliosis and vitiligo and a diagnosis of complete VKH with APECED was made.
Conclusion(s) APECED is a rare endocrine disorder and has been reported to be associated with VKH twice. Likewise, VKH is commonly present in much older patients; this is the first time ever to be diagnosed in a two-year-old child.
