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Dowling-Degos Disease: Case Report and Review of the Literature

Batycka-Baran A.a · Baran W.a · Hryncewicz-Gwozdz A.a · Burgdorf W.b
aDepartment of Dermatology, Venereology and Allergology, Wroclaw Medical University, Wroclaw, Poland; bDepartment of Dermatology, Ludwig Maximilian University, Munich, Germany Dermatology 2010;220:254–258 (DOI:10.1159/000278349)


Dowling-Degos disease (DDD) is an unusual pigmentary disorder usually caused by mutations in keratin 5. A 44-year-old woman in good general health presented due to the recent appearance of numerous pigmented macules on her axillary and anogenital skin. A biopsy showed lacy, finger-like epidermal extensions into the dermis which were heavily pigmented and associated with tiny cysts or dilated follicles. We view DDD as part of a spectrum of disorders which are morphologically related but vary in location and time of expression. In addition, both the clinical and histological differential diagnostic considerations are extensive.


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