Gronblad-Standberg syndrome: a case report and review of literature
Arfan ul Bari, Inam Ul Haq, Tahir Mumtaz Malik
Abstract
Arfan ul Bari, Inam Ul Haq, Tahir Mumtaz Malik
Abstract
Gronblad-Strandberg syndrome is a rare congenital hereditary dysplasic disorder of the connective tissue, characterized by a progressive abnormal mineralization and dystrophic calcification of elastic tissue and collagen. The term Gronblad-Strandberg syndrome is used when angioid streaks in eye occur in association with pseudoxanthoma elasticum (PXE) of skin. We report a young patient having characteristic skin and eye manifestations of the syndrome. He was evaluated thoroughly regarding extent and potential systemic complications of the disease and was counseled accordingly. Since dermatologists are generally not familiar with historical background and ocular findings of the disease, a brief review of these two aspects is also made.
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Gronblad-Strandberg syndrome is a rare congenital hereditary dysplasic disorder of the connective tissue, characterized by a progressive abnormal mineralization and dystrophic calcification of elastic tissue and collagen. The term Gronblad-Strandberg syndrome is used when angioid streaks in eye occur in association with pseudoxanthoma elasticum (PXE) of skin. We report a young patient having characteristic skin and eye manifestations of the syndrome. He was evaluated thoroughly regarding extent and potential systemic complications of the disease and was counseled accordingly. Since dermatologists are generally not familiar with historical background and ocular findings of the disease, a brief review of these two aspects is also made.
Key concepts: Pseudoxanthoma elasticum, Medicine, Angioid streaks, Dermatology, Connective Tissue Disorder, Calcification, Dystrophic calcification, Disease