There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.
Abstract
Dowling-Degos disease (DDD) is a rare inherited disease characterized by reticular
hyperpigmentation on flexor surfaces.
We sought to describe several cases of generalized DDD, a presentation that resemble
dyschromatosis universalis hereditaria.
The clinical manifestations, histopathologic, and genetic studies of a family with
autosomal dominant inheritance were analyzed.
The father and his sister had reticular hyperpigmentation on flexor surfaces, whereas
the daughter and son had generalized hyperpigmentation with numerous hypopigmented
or erythematous macules and papules on the trunk and limbs. Skin biopsy specimens
from both types of lesions all had typical features of DDD. Biopsy specimens from
axillary skin had features of Galli-Galli disease, an acantholytic form. There were
no mutations of the double-stranded RNA-specific adenosine deaminase or keratin 5
genes.
Generalizations cannot be drawn from genetic study of only one family.
DDD can present with generalized hyperpigmentation and hypopigmented papules.