[Macroreticular and butterfly shaped dystrophies of the retinal pigment epithelium (author's transl)].
We report the observation of a familiy with 4 cases of macroreticular dystrophy (Mesker) and 1 case of butterfly shaped dystrophy (Deutman) in its ranks. Diagnosis of these conditions is based on ophtalmoscopic and angiographic appearence. Visual loss may be somewhat important in the macroreticular type and it may increase as time passes on. Transmission is autosomal dominant with variable penetrance. The fact that there are anomalies of the retinal pigment epithelium in periphery and around the disk in three of our cases leads us to think that this is a diffuse disease of the retinal pigment epithelium. Macroreticular and butterfly shaped dystrophies are probably similar diseases, they form altogether with the reticular dystrophies (Sjögren) the group of the patterned dystrophies of the retinal pigment epithelium.
D000328:Adult; D002648:Child; D004585:Electrooculography; D004596:Electroretinography; D005260:Female; D005451:Fluorescein Angiography; D005654:Fundus Oculi; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D010857:Pigment Epithelium of Eye / Q000473:pathology*; D012162:Retinal Degeneration / Q000175:diagnosis* / Q000235:genetics; D014792:Visual Acuity
Dystrophies en réseau de l'épithélium pigmentaire (dystrophies macroréticulaires et en aile de papillon).