[Doyne's macular heredodystrophy and benign monoclonal gammopathy. Genetic and pathogenetic correlations (author's...

Doyne's macular heredodystrophy associated with benign monoclonal gammopathy was seen in 4 patients having genetic correlations in 3 generations. One case showed crystalline stromal corneal deposits. Based on this pathological association and the morphofunctional support of the pair, choriocapillaris Bruch's membrane, Doyne's macular heredodystrophy is considered as a symptom of the immunoglobulin deficiency syndrome (IgM). The pathogenicity of Doyne's macular dystrophy and of the secondary drusen is interpreted as a dynamic phenomenon related to all the morphofunctional changes occurring at the level of the choriocapillaris Bruch's membrane, and the pigmented epithelium.