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August 1964

Epidermolysis Bullosa Acquisita, Dermal Elastosis, Amyloidosis

Author Affiliations


From the Division of Dermatology, Department of Medicine and the Center for the Study of Aging, Duke University Medical Center.

Arch Dermatol. 1964;90(2):137-142. doi:10.1001/archderm.1964.01600020005001

A patient is described with epidermolysis bullosa acquisita, primary amyloidosis (which did not involve the skin), and elastosis in the areas of skin affected by epidermolysis but not in the normal skin. The elastosis was confirmed by biochemical methods. However, the amino acid composition of the isolated elastin was unusual. There was a decrease in the insoluble collagen fraction and an increase in total hexosamine. Because the demonstration of increased elastic tissue and acid mucopolysaccharide histochemically differs sharply from observations of other workers, it is believed that this case may represent a new disease entity with clinical epidermolysis bullosa acquisita and biochemically proved elastosis associated with primary amyloidosis.

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