Ann Dermatol.  2011 May;23(2):205-208. 10.5021/ad.2011.23.2.205.

A Case of Dowling-Degos Disease on the Vulva

• Kang HS
• Hur J
• Lee JW
• Oh DH
• Yeo KY
• Kim JS
• Yu HJ

• KMID: 2156664
• DOI: http://doi.org/10.5021/ad.2011.23.2.205

Abstract

Dowling-Degos disease (DDD) is an autosomal dominant genodermatosis and this disease is a genetically determined disturbance of epidermal proliferation. It is characterized by acquired, slowly progressive pigmented lesions that primarily involve the great skin folds and flexural areas such as the axilla, neck, limb flexures, the inframammary area and the inguinal folds. The vulva is an unusual location for DDD. A 41-year-old woman presented with a 10-year history of multiple, small, reticulated and brownish macules distributed symmetrically on the bilateral external genital regions. We found no other similarly pigmented skin lesions on her body, including the flexural areas. There was no known family history of similar eruptions or pigmentary changes. The histologic examination showed irregular rete ridge elongation with a filiform or antler-like pattern and basilar hyperpigmentation on the tips. Fontana-Masson staining showed increased pigmentation of the rete ridges and the S100 protein staining did not reveal an increased number of melanocytes in the epidermis. From these findings, we diagnosed this lesion as DDD.

Keyword

Dowling-Degos disease; Pigmented skin lesion; Vulva

MeSH Terms

Adult
Axilla
Dichlorodiphenyldichloroethane
Epidermis
Extremities
Female
Humans
Hyperpigmentation
Melanocytes
Neck
Pigmentation
Skin
Skin Diseases, Genetic
Skin Diseases, Papulosquamous
Vulva
Dichlorodiphenyldichloroethane
Hyperpigmentation
Skin Diseases, Genetic
Skin Diseases, Papulosquamous

Figure

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  Fig. 1 (A) Multiple hyperpigmented brownish macules located symmetrically on the bilateral external genital area. (B) A close up view of the reticulate pigmented lesion.

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  Fig. 2 (A) The epidermal downgrowths showed a filiform or antler-like pattern with irregular elongation of the rete ridges in the lesion (H&E, ×100). (B) There is increased melanin pigment at the tips of the epidermis (H&E, ×200). (C) There is increased pigmentation of the rete ridges on Fontana-Masson staining (Fontana-Masson, ×200). (D) Compared with perilesional normal skin, the S100 protein staining reveals no increase in the number of melanocytes (S100 protein, ×200).

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