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

Four cases of Dowling Degos disease (DOD) in one family and five cases of Haber's syndrome (HS) in two families were compared. nOD 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 DOD had basal cell epithelioma within the area of reticular pigmentation, but an 83year-old patient with DOD indicated that general health is not involved in DOD.One differentiating point for HS is that black warts in HS do not have the histologic features of DDQ.; 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 ODD, characterized by red facies, seborrheic keratosis, and earlier onset than DOD. Both DDD and HS appear to be conditions with a different reactivity of the melanoeyte-keratinocyte system to external stimulation, rather than a disease of nevoid origin.

Dowling-Degos disease associated with squamous cell carcinomas on the dappled pigmentation

“…Our patient revealed characteristic pigmentation uniquely localized to the buttocks, in contrast to the previously reported patients with Dowling–Degos disease who mainly manifested reticular pigmentation of the flexural areas even though they showed hyperpigmentation on the buttock 1,3 , 7 . This pigmentation was not clearly reticulate but rather dappled as pointed out by Wilson Jones and Grice, 1 who noted that less severely affected lesions of Dowling–Degos disease show dappled patterns in varying shades of brown.…”

“…Our patient revealed characteristic pigmentation uniquely localized to the buttocks, in contrast to the previously reported patients with Dowling-Degos disease who mainly manifested reticular pigmentation of the flexural areas even though they showed hyperpigmentation on the buttock. 1,3,7 This pigmentation was not clearly reticulate but rather dappled as pointed out by Wilson Jones and Grice, 1 who noted that less severely affected lesions of Dowling-Degos disease show dappled patterns in varying shades of brown. The other clinical findings consistent with Dowling-Degos disease were comedone-like lesions on the face and back, a finger-like fibroma in the popliteal fossa, dystrophic fingernails, and a large number of seborrhoeic keratosis-like lesions.…”

“…Dowling–Degos disease (reticulate pigmented anomaly of the flexures 1 ) is an autosomal dominant genodermatosis characterized by an epithelial naevoid anomaly 2 showing reticular pigmentation of the flexural areas and follicular lesions such as pitted scars near the angles of the mouth and dark comedone‐like lesions on the neck. In addition to these features, multiple seborrhoeic keratosis‐like lesions, 1,3–6 finger‐like fibroma, 2,7 dystrophic fingernails 8,9 and follicular lesions such as epidermoid cysts, 1,5 , 7 pilonidal sinus 1,7 and hidradenitis suppurativa 10–13 are often associated. Histological characteristics are narrow strands of heavily pigmented epithelial cells extending from both the epidermis and pilosebaceous follicles.…”

Evolution of Dowling Degos Disease

“…To the Editor: With great interest I read "A case of Dowling-Degos disease suggesting/an evolutional sequence" which appeared in J Dermatol, 27: 591-597, 2000 (I). It reminded me of our paper "The broad spectrum of Dowling Degos disease including Haber's syndrome -A hereditary abnormal reactivity to stimulation, increasing with age?-Case reports and management" which appeared in J Dermatol, 10: 361-375, 1983 (2).…”