Dowling Degos Disease
H. Gammaz, H. Amer, F. Ibrahim & M. Bagdady
Egyptian Dermatology Online Journal 6 (1): 11AL- Haud Al- Marsoud Hospital, Cairo, Egypt
Submitted: April 15, 2010
Accepted: May 1, 2010
A 30 years old female presented with small, rounded, pigmented macules that resemble freckles which were affecting her face, infra-mammary regions and axillae. Histopathological examination revealed hyperkeratosis, acanthosis, papillomatosis and hype-rmelanosis.
Dowling Degos disease is a rare condition inherited via an autosomal dominant gene. It presents during adult life most frequently in the 4th decade as numerous, small, rounded pigmented macules that resemble freckles. The axillae and groins are the usual sites, although other areas may be involved including inter-gluteal folds, infra-mammary folds, neck, scalp, trunk and arms . Involvement of genitalia has been described . Pigmentation is symmetrical and progressive but otherwise asymptomatic .
A 30 years old female presented to our outpatient clinic at Al-Haud Al-Marsoud Hospital complaining of progressive small, rounded, pigmented macules that resemble freckles. The lesions were found affecting her face (fig. 1), both infra-mammary regions (fig. 2) and axillae (fig. 3). On examination, we found comedo like lesions, pitted acneform scars near the angles of the mouth (fig. 4).
Our differential diagnoses included Dowling Degos disease, acanthosis nigricans, multiple basal cell papillomas, epidermal nevi and axillary freckles of neurofibramatosis.
Histopathological examination of the specimen which was stained by Haematoxylin & Eosin (fig. 5-7) revealed hyperkeratosis, acanthosis, papillomatosis and hypermelanosis. Also there was branching heavily melanizied epidermal downward proliferation arising from the lower border of the epidermis and small horn cysts were found within the epidermal proliferation. The histopathological findings confirmed that we were dealing with a case of Dowling Degos disease.
In Dowling Degos disease, the degree of pigmentation varies, but in some patients the lesions are almost confluent giving a brown or black lace like pattern . Other reported features are comedo- like lesions (dark dot follicles) and pitted acneform scars occurring near the angle of the mouth .
Reported associations of the disease are hidradinitis suppurativa, mental retardation or trichilemmal cysts .
It has been suggested that Dowling Degos disease is one part of a spectrum of conditions that are characterized by reticulate pigmentation, this would include some autosomal dominant conditions as Kitamura`s acropigmentation reticularis .
Successful treatment of Dowling Degos disease with Er: YAG laser pulse energy between 1000 and 1200 mJ three consecutive passes leads to good results .
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