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Jan 2012

Reticulate Hyperpigmentation of the Vulva —Diagnosis

Arch Dermatol. 2012;148(1):113-118. doi:10.1001/archderm.148.1.114-d

Histopathological analysis (Figure 3) revealed filiform elongation of epidermal rete ridges, with prominent pigmentation of basal keratinocytes and some melanin incontinence. Close clinicopathologic correlation led to the diagnosis of DDD.

Dowling-Degos disease is an autosomal dominant genodermatosis, presenting with reticulate pigmentation of flexures, genital folds, and pectoral skin. There may be comedolike, black follicular hyperkeratotic papules and pitted perioral scars. The condition causes pruritus and is aesthetically disfiguring but otherwise asymptomatic. It is more common in women, presenting in adult life —mostly the fourth decade. Some cases have been associated with furunculosis of the axillae, obesity, diabetes mellitus, hypertension, pregnancy,1 and hidradenitis suppurativa.2 Pathogenesis is unknown, but loss-of-function mutations in the keratin-5 gene (chromosome 12q) have been identified.

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