7 Nov Me llamo Loli tengo 37 años y vivo en Madrid y me diagnoticaron la enfermedad de Degos en despues de que perdiera la vision del ojo. Se describe el caso de una mujer de 60 años de edad que presentaba desde hacía años lesiones hiperpigmentadas reticuladas que afectaban a los pliegues . Enfermedad de Dowling-Degos: caso clínico asociado a hidradenitis supurativa. Dowling-Degos Disease Associated With Hidradenitis Suppurativa: A Case.
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In early stages, histology of lesions may reveal a superficial and deep perivascular lymphocytic infiltration with distinct mucin deposition. Management and treatment Therapeutic efforts with anticoagulants and compounds that facilitate blood perfusion, such as acetylosalicylic acid, pentoxifylline, dipyridamole, ticlodipine and heparin have achieved a partial regression defos skin lesions in some individual cases.
Systemic manifestations are progressive and may lead to serious complications: A clinical inspection of the skin should be combined with additional examinations including brain magnetic resonance dee, gastroscopy, colonoscopy, X-ray of the chest and abdominal ultrasound, in order to assess the long-term prognosis.
Diagnosis is based primarily on the cutaneous clinical picture that is nearly pathognomonic. The histology of early lesions resembles cutaneous lupus erythematosus enfermrdad this term. Hypotheses implicating vasculitis, coagulopathy or a primary dysfunction of endothelial cells have been proposed.
If you have questions about the archived correspondence, please use the OTRS noticeboard. No effective treatment for the systemic manifestations has been established, however, enfeermedad treprostinil has been tested successfully in one case with intestinal and CNS manifestations. Retrieved from ” https: I, the copyright holder of this work, hereby publish it under the re license:. Other search option s Alphabetical list. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.
Thomas Habif authorises the use of the 21 pictures listed here and published on the website dermnet. Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. Permission Reusing this file. This correspondence has been reviewed by an OTRS member and stored in our permission archive.
Systemic lesions may affect the gastrointestinal tract and the central nervous system CNS and are potentially lethal. Wikimedia has received an e-mail confirming that the copyright holder has approved publication under the terms mentioned on this page. MAP onset occurs in adults aged with skin lesions that appear initially as small erythematous papules, predominantly on the trunk and the upper extremities. The documents contained in this web site are presented for information purposes only.
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enfermedad de Degos – English Translation – Word Magic Spanish-English Dictionary
More developed lesions show prominent changes in the dermoepidermal junction, with atrophy of the epidermis and an area of sclerosis in the papillary dermis. Therapeutic efforts with anticoagulants and compounds that facilitate blood perfusion, such as acetylosalicylic acid, pentoxifylline, dipyridamole, ticlodipine and heparin have achieved a partial regression of skin lesions in some individual cases.
Idiopathic, monosymptomatic, cutaneous presentations are benign, however, systemic manifestations can develop years after the occurrence of skin lesions. Disease definition Malignant atrophic papulosis MAP is a rare, chronic, thrombo-obliterative vasculopathy characterized by papular skin lesions with central porcelain-white atrophy and a surrounding teleangiectatic rim.
This work is free and may be used by anyone for any purpose. The etiopathogenesis of the disease remains unknown.
Later a wedge-shaped connective tissue necrosis in the deep dermis, due to a thrombotic occlusion of the small arteries and sparse lymphocytes, occurs. Specialised Social Services Eurordis directory.
Enfermedad de Dowling-Degos – ScienceDirect
Check this box if you wish to receive a copy of your message. Diagnostic methods Diagnosis is based primarily on the cutaneous clinical picture that is nearly pathognomonic.
From Wikimedia Commons, the free media repository. Genetic counseling A genetic predisposition with an autosomal dominant degow has been suggested. For all other comments, please send your remarks via contact us.
This page was last edited on 25 Septemberat Views View Edit History. Degos disease Date Source http: Ocular involvement, which affects the eyelids, conjuctiva, retina, sclera and the choroid plexus, as well as the development of diplopia and ophthalmoplegia as secondary side effects of the detos involvementhas also been described.
Many patients have been reported to have defects in blood coagulation. Degos disease Injured human backs.
Additional information Further information on this disease Classification s 3 Gene s 0 Clinical signs and symptoms Publications in PubMed Other website s 7.
Summary [ edit ] Description Degos-disease. Health care resources for this disease Expert centres 65 Diagnostic tests 0 Patient organisations enferemdad Orphan drug s 0. As all patients may potentially develop the systemic, life-threatening variant, an annual follow-up is mandatory.
The following other wikis use this file: Summary and related texts. Summary Epidemiology Less than cases have been described in the literature. More developed lesions can imitate lichen sclerosus see this term.
It may more rarely manifest as pericarditis or in other organs such as the lungs, presenting as pleuritis. Only comments written in English can be processed.