Vitelliform macular dystrophy and butterfly-shaped epithelial dystrophy: a continuum? Br i Ophthalmol

SUMMARY We report the case of a 22-year-old white female who presented for a routine ocular examination and displayed Best's disease in her right eye and butterfly-shaped macular dystrophy in her left eye. These diagnoses were confirmed by fluorescein angiography, EOG, and ERG. The fact that these 2 disorders are present in one person suggests that they are only different manifesta-tions of the same underlying disease process. Vitelliform macular dystrophy (Best's disease) and butterfly-shaped epithelial dystrophy of the macula (Deutman's dystrophy) have been described as 2 separate heredodystrophic diseases affecting the retinal pigment epithelium and the outer retina. 2 We report a patient who has vitelliform macular Fig. 1 Fundus photograph OD: An oval, well-demarcated, yellowish, macular lesion, 3/4 disc diameter in size. dystrophy (VMD) in the right eye and butterfly-shaped epithelial dystrophy (BED) in the left eye. This is the first case that suggests these 2 rare Correspondence to 1. Gutman, MD. diseases may be different manifestations of the same heritable disorder. Case report The patient is a 22-year-old white female who Fig.2 Fundus photograph OS: A butterfly-shaped, hyperpigmented lesion, about 3 disc diameters in size, in the macular region. presented to the eye clinic for a routine examination. She had no visual complaints. Medical and ocular histories were unremarkable. Ocular examination showed an uncorrected visual acuity of 6/6 (20/20