title: Dowling-Degos disease involving the vulva and back: case report and review of the literature.
abstract: Dowling-Degos disease is a rarely encountered pigmentary disorder in which small brown-to-black macules appear in a clustered or reticulated pattern primarily at flexural sites. It usually occurs as an autosomal dominant trait but sporadic cases have also been reported. Dowling-Degos disease is sometimes associated with other cutaneous abnormalities, many of which appear to occur as a result of abnormal follicular development. The histology is distinctive with marked, heavily pigmented, slender, and often branched, elongation of the rete ridges. Dowling-Degos disease is caused by one of several loss-of-function mutations in the keratin 5 gene. Similar mutations are found in patients with Galli-Galli disease and that disorder is now considered to be a subset of Dowling-Degos disease. Medical therapy is ineffective but two patients have responded well to ablative laser therapy. We report a patient with the sporadic form of the disease who developed pigmented macules in the rarely involved sites of the lower back and vulva. Her vulvar lesions were treated with Er:YAG laser ablation.
authors.affiliation: University of California, Davis School of Medicine, Sacramento, California, USA.
authors.forename: Mary E
authors.initials: ME
authors.lastname: Horner
authors.forename: Katherine E
authors.initials: KE
authors.lastname: Parkinson
authors.forename: Valda
authors.initials: V
authors.lastname: Kaye
authors.forename: Peter J
authors.initials: PJ
authors.lastname: Lynch
mesh_terms: D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D053555:Keratin-5 / Q000235:genetics; D053844:Lasers, Solid-State / Q000627:therapeutic use; D008161:Lumbosacral Region / Q000473:pathology*; D009154:Mutation; D010859:Pigmentation Disorders / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D014844:Vulva / Q000473:pathology*; D055815:Young Adult
chemical_list: D053555:Keratin-5