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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.
AuthorsMary E Horner, Katherine E Parkinson, Valda Kaye, Peter J Lynch
JournalDermatology online journal (Dermatol Online J) Vol. 17 Issue 7 Pg. 1 (Jul 15 2011) ISSN: 1087-2108 [Electronic] United States
PMID21810386 (Publication Type: Case Reports, Journal Article, Review)
Chemical References
  • Keratin-5
Topics
  • Adult
  • Diagnosis, Differential
  • Female
  • Humans
  • Keratin-5 (genetics)
  • Lasers, Solid-State (therapeutic use)
  • Lumbosacral Region (pathology)
  • Mutation
  • Pigmentation Disorders (genetics, pathology, surgery)
  • Vulva (pathology)
  • Young Adult

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