Generalized Dowling-Degos disease

Department of Dermatology, Mackay Memorial Hospital, Taipei, Taiwan.
Journal of the American Academy of Dermatology (Impact Factor: 4.45). 09/2007; 57(2):327-34. DOI: 10.1016/j.jaad.2006.12.023
Source: PubMed


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.

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    ABSTRACT: Dowling-Degos disease (DDD) is a rare autosomal dominant trait characterized by numerous, symmetrical, progressive and pigmented macules over the axillae, groins, face, neck, arms and trunk as well as scattered comedo-like lesions (dark dot, follicles) and pitted acneiform scars. Histopathology is diagnostic testing using a distinctive form of acanthosis, characterized by an irregular elongation of thin branching rete ridges, with a concentration of melanin at the tips. We report cases of generalized DDD in a single family with autosomal dominant penetrance. DDD can be presented in a generalized form with hypopigmented lesions instead of reticulate hyperpigmentation confined to the flexor areas. This form can be differentiated from DUH by histopathology.
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