Generalized Dowling-Degos disease - 19/08/11
, Yang-Chih Lin, MD a, b, c
Reprint requests: Yu-Hung Wu, MD, Department of Dermatology, Mackay Memorial Hospital, 92, Sec 2, Chung-Shan N Rd, Taipei, Taiwan, 10449.Taipei, Taiwan
Abstract |
Background |
Dowling-Degos disease (DDD) is a rare inherited disease characterized by reticular hyperpigmentation on flexor surfaces.
Objective |
We sought to describe several cases of generalized DDD, a presentation that resemble dyschromatosis universalis hereditaria.
Methods |
The clinical manifestations, histopathologic, and genetic studies of a family with autosomal dominant inheritance were analyzed.
Results |
The father and his sister had reticular hyperpigmentation on flexor surfaces, whereas the daughter and son had generalized hyperpigmentation with numerous hypopigmented or erythematous macules and papules on the trunk and limbs. Skin biopsy specimens from both types of lesions all had typical features of DDD. Biopsy specimens from axillary skin had features of Galli-Galli disease, an acantholytic form. There were no mutations of the double-stranded RNA-specific adenosine deaminase or keratin 5 genes.
Limitation |
Generalizations cannot be drawn from genetic study of only one family.
Conclusion |
DDD can present with generalized hyperpigmentation and hypopigmented papules.
Il testo completo di questo articolo è disponibile in PDF.Abbreviations used : DDD, DSH, DSRAD, DUH, KRT5, RAPK
Mappa
| Supported by the Mackay Memorial Hospital grant MMH-E-95004. Conflicts of interest: None declared. |
Vol 57 - N° 2
P. 327-334 - agosto 2007 Ritorno al numero
Articolo precedente
- Intractable wounds caused by arteriosclerosis obliterans with end-stage renal disease treated by aggressive debridement and epidermal grafting
- Takaaki Hanafusa, Yuji Yamaguchi, Ichiro Katayama
- Treatment of acute Old World cutaneous leishmaniasis: A systematic review of the randomized controlled trials
- Alireza Khatami, Alireza Firooz, Farzam Gorouhi, Yahya Dowlati
Benvenuto su EM|consulte, il riferimento dei professionisti della salute.
L'accesso al testo integrale di questo articolo richiede un abbonamento.
Già abbonato a @@106933@@ rivista ?