Dowling-Degos disease (DDD) is a rare dominant inherited genodermatosis characterized clinically by a reticular pigmentation of the skin folds, small comedo-like hyperkeratotic follicular lesions and pitted acneiform scars generally around the mouth. Histological study reveals epidermal proliferation with fingerlike downgrowth: large amounts of melanin are present particularly in the basal layer, but melanocytes are not altered in appearance or in number. The elongation of rete ridges involves also the follicular infundibulum with, sometimes, follicular plugging. In this paper the authors present a study of seven cases of DDD, 1 man and 6 women, 30-56 years of age, observed in 7 years. All these patients showed reticular pigmentation of the folds; in six cases there were also pitted acneiforms scars and in five cases comedo-like lesions. In particular, in a woman the reticulate pigmented anomaly involved the dorsum of the hands. Four of seven patients showed association with hidradenitis suppurativa. A family history revealed that 14 members of 6 families were affected with variable clinical expression of the disease. Sometimes, the typical lesions were soft and only the histological picture permitted diagnosis. The authors emphasize the importance, in patients affected by DDD, of a careful family history and identification of minimal clinical expressivity forms.
|Translated title of the contribution||Dowling Degos disease: Study of seven cases observed in Central and Southern Italy|
|Number of pages||4|
|Journal||Giornale Italiano di Dermatologia e Venereologia|
|Publication status||Published - 1994|
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