Generalized Dowling-Degos disease

Department of Dermatology, Mackay Memorial Hospital, Taipei, Taiwan.
Journal of the American Academy of Dermatology (Impact Factor: 4.45). 09/2007; 57(2):327-34. DOI: 10.1016/j.jaad.2006.12.023
Source: PubMed


Dowling-Degos disease (DDD) is a rare inherited disease characterized by reticular hyperpigmentation on flexor surfaces.
We sought to describe several cases of generalized DDD, a presentation that resemble dyschromatosis universalis hereditaria.
The clinical manifestations, histopathologic, and genetic studies of a family with autosomal dominant inheritance were analyzed.
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.
Generalizations cannot be drawn from genetic study of only one family.
DDD can present with generalized hyperpigmentation and hypopigmented papules.

Download full-text


Available from: Yu-Hung Wu, Oct 07, 2015
62 Reads
  • Source
    • "In order to categorize these different disorders with overlapping clinical or histopathologic features, it seems that there are two major groups20. The first is DDD, which has the characteristic histologic findings of elongated rete ridges, horn cyst formation and hyperpigmented tips. "
    [Show abstract] [Hide abstract]
    ABSTRACT: Dowling-Degos disease (DDD) is a rare autosomal dominant trait characterized by numerous, symmetrical, progressive and pigmented macules over the axillae, groins, face, neck, arms and trunk as well as scattered comedo-like lesions (dark dot, follicles) and pitted acneiform scars. Histopathology is diagnostic testing using a distinctive form of acanthosis, characterized by an irregular elongation of thin branching rete ridges, with a concentration of melanin at the tips. We report cases of generalized DDD in a single family with autosomal dominant penetrance. DDD can be presented in a generalized form with hypopigmented lesions instead of reticulate hyperpigmentation confined to the flexor areas. This form can be differentiated from DUH by histopathology.
    Annals of Dermatology 08/2013; 25(3):360-4. DOI:10.5021/ad.2013.25.3.360 · 1.39 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Introspection leads us to believe that universals and meaning are extracted from sensory information before responsive action commences: as if we act first as passive observers whose subsequent reactions follow a process of recognition and decision. We are inclined to base our designs of cognitive systems upon such subjective insights. However, if we take into account the necessity for a prior, ontogenetic growth of a repertoire of perception, it appears more probable that we are engaged in unceasing interaction with our environment and that a response itself, driven by homeostatic mechanisms, contains the meaningful information about the object of the response. The components of the world are thereby described not in terms of their intrinsic spatio-temporal parameters but by their relation to the observer. A rudimentary visual system employing a Probability State Variable (PSV) Controller as a, homeostat is described, and the prototype peripheral hardware suggested. The addition of an associative memory would allow the buildup of a cognitive model of the world, but the reactive searching by the system described --- as if it were an autonomous "perceptuo-postural" mechanism --- must begin the process of discovering the properties of reality.
    Adaptive Processes, Sixth Symposium on; 01/1967
  • [Show abstract] [Hide abstract]
    ABSTRACT: Analyses of historical contestations over the meanings of “expertise” and its impact on agricultural development are presented. The paper illustrates the power struggle between competing groups to define expertise and to then use these experts to maintain their economic dominance. The paper also examines current and potential challenges to the political and material sustainability of biotechnology driven development programs given the increasingly contested nature of knowledge. The roles of research scientists, women's organizations and extension agents in this struggle are emphasized
    Technology and Society Technical Expertise and Public Decisions, 1996. Proceedings., 1996 International Symposium on; 07/1996
Show more