Dowling-Degos Disease, also known as reticulate acropigmentation of Kitamura or reticular pigmented anomaly of flexures, is a rare benign genodermatosis. Differentiation from acanthosis nigricans is important because of its favorable prognosis. Onset is most often in the 30- to 40-year age range. The disease is slowly progressive, but not life threatening. The anomaly initially affects the axillae and groins. Flexural pigmented reticulate macules and sometimes comedo-like papules occur on the back and/or the neck. Some patients have pitted perioral scars. Pruritus of affected flexural areas may be the only symptom. In both male and female patients, pigmented reticulate macules may also be evident on the genitalia. Later in life the intergluteal and inframammary folds, neck, trunk and arms may be involved.
Heterozygous mutations in the keratin-5 (KRT5) gene have been reported as the genetic cause of Dowling-Degos Disease.