LETTER TO JMG Identification of novel locus for autosomal dominant butterfly

utterfly shaped macular dystrophy was first described by Deutman et al. in 1970.1 It is characterised by bilateral accumulation of pigmented or yellowish material at the level of the retinal pigment epithelium. Lesions consist of 3–5 ‘‘wings,’ ’ which resemble the wings of a butterfly. Affected patients present with a subnormal electrooculogram and normal or slightly diminished visual acuity. The disease is relatively benign, but it can progress with age to chorioretinal atrophy in the parafoveal and peripapillary regions.2 Butterfly shaped macular dystrophy shares impor-tant similarities with age related macular degeneration—the most common cause of blindness in older patients.3 4 In both diseases, abnormal deposition of lipofuscin like material at the level of the retinal pigment epithelium is found, which results in loss of the overlying photoreceptors.