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Dermatofibrosis Lenticularis Disseminata With Osteopoikilosis

Carl J. Raque, MD; Margaret Gray Wood, MD

Arch Dermatol. 1970;102(4):390-396.

Abstract
Osteopoikilosis is a rare disease of the bone that is occasionally associated with skin lesions. The latter have been reported as dermatofibrosis lenticularis disseminata, juvenile elastoma, and connective-tissue nevus. The occurrence of this syndrome in three members of a family is used to demonstrate the salient features of familial incidence, characteristic distribution patterns, and histologic variations. The clinical similarities between variously reported cases and recent work at the ultrastructural level offer an explanation of the histologic variations found in these lesions. There is confusion associated with the nomenclature and a classification is offered that permits the identification of dermatofibrosis lenticularis disseminata as a type of connective-tissue nevus.

Author Affiliations
Philadelphia

From the Department of Dermatology, University of Pennsylvania School of Medicine, Philadelphia.

Footnotes
Accepted for publication May 25, 1970.

Reprint requests to Department of Dermatology, Duhring Laboratories, Hospital of the University of Pennsylvania, Philadelphia 19104 (Dr. Raque).

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