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.
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Authors | Mary E Horner, Katherine E Parkinson, Valda Kaye, Peter J Lynch |
Journal | Dermatology online journal
(Dermatol Online J)
Vol. 17
Issue 7
Pg. 1
(Jul 15 2011)
ISSN: 1087-2108 [Electronic] United States |
PMID | 21810386
(Publication Type: Case Reports, Journal Article, Review)
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Chemical References |
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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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