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Generalized Dowling-Degos disease

Published on Aug 1, 2007in Journal of The American Academy of Dermatology7.10
· DOI :10.1016/j.jaad.2006.12.023
Yu-Hung Wu2
Estimated H-index: 2
(Mackay Memorial Hospital),
Yang-Chih Lin1
Estimated H-index: 1
(Mackay Memorial Hospital)
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.
  • References (32)
  • Citations (45)
References32
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#1Cheng-Rang Li (Nanjing Medical University)H-Index: 1
#2Qinghe Xing (SJTU: Shanghai Jiao Tong University)H-Index: 26
Last.Wenyuan Zhu (Nanjing Medical University)H-Index: 4
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#1S‐C. Chao (NCKU: National Cheng Kung University)H-Index: 8
#2Jun-Young Lee (NCKU: National Cheng Kung University)H-Index: 22
Last.M‐H. Yang (NCKU: National Cheng Kung University)H-Index: 3
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#1Yong Cui (Wannan Medical College)H-Index: 26
#2Jun Wang (Wannan Medical College)H-Index: 1
Last.X. Zhang (Anhui Medical University)H-Index: 68
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#1X. Zhang (Anhui Medical University)H-Index: 68
#2Ping-Ping He (Anhui Medical University)H-Index: 14
Last.Jian-Jun Chen (Anhui Medical University)H-Index: 14
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#1J. Knuever (University of Cologne)
#2Oana‐Diana Persa (University of Cologne)H-Index: 2
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#1Wanting Yu (Academy of Medical Sciences, United Kingdom)
#2Lu Gan (Academy of Medical Sciences, United Kingdom)
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#1Jian Zhang (SJTU: Shanghai Jiao Tong University)H-Index: 11
#2M. Li (SJTU: Shanghai Jiao Tong University)H-Index: 4
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#1Thuy L. Phung (BCM: Baylor College of Medicine)H-Index: 17
#2Teresa S. Wright (UTHSC: University of Tennessee Health Science Center)
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#1Jian Zhang (SJTU: Shanghai Jiao Tong University)H-Index: 11
#2Ruhong Cheng (SJTU: Shanghai Jiao Tong University)H-Index: 10
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#1Robert H. Sorensen (San Antonio Military Medical Center)H-Index: 1
#2Kimberly A. Werner (San Antonio Military Medical Center)H-Index: 1
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View next paperGalli–Galli disease is an acantholytic variant of Dowling–Degos disease