DOWLING-DEGOS DISEASE

By Gary M. White, MD


Dowling-Degos disease (DDD), also known as "reticulate pigmented anomaly of the flexures," is a rare, reticulated pigmentary disorder usually caused by mutations in keratin 5.

Clinical

Reticulated hyperpigmented macules occur in the axillary, inguinal, and inframammary areas. Rarely, the wrists, face, scalp, scrotum, and vulva may be affected. Comedone-like lesions, pruritus, and pitted perioral scars are common features [JAAD 1999;40;462]. Patients often come in complaining of the comedones.

Treatment

Topical adapalene gel BID was quite helpful in one case [Dermatology 1999;198;176]. Topical tretinoin, hydroquinone, topical steroids, and systemic retinoids have been tried but are usually ineffective.

References

A Case of Dowling-Degos Disease on the Vulva. Ann Dermatol. 2011 May;23(2):205-208

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