The broad spectrum of Dowling Degos disease, including Haber's syndrome--a hereditary abnormal reactivity to stimulation, increasing with age?--Case reports and management.

Abstract

ABSTRACTFour cases of Dowling Degos disease (DDD) in one family and five cases of Haber's syndrome (HS) in two families were compared. DDD and HS share many symptoms and findings in common; histopathological features of downgrowths of epithelial cells, papules of either brown or natural skin color, and various kinds of skin pigmentation. Some had pitted scars, cysts, pruritus, telangiectasia, red cheeks, xerosis, and photosensitivity. Another feature common to both conditions is the progressive course of the disease; patients and their children should be followed over their lifetimes. One case of DDD had basal cell epithelioma within the area of reticular pigmentation, but an 83‐year‐old patient with DDD indicated that general health is not involved in DDD.One differentiating point for HS is that black warts in HS do not have the histologic features of DDD; they show only seborrheic keratosis. In HS, microscopic examinations should be made of as many varied lesions as possible.HS appears to be a subtype of DDD, characterized by red facies, seborrheic keratosis, and earlier onset than DDD. Both DDD and HS appear to be conditions with a different reactivity of the melanocyte‐keratinocyte system to external stimulation, rather than a disease of nevoid origin.