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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Idiopathic, monosymptomatic, cutaneous presentations are benign, however, systemic manifestations can develop years after the occurrence of skin lesions. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis enfrrmedad diagnosis or treatment. The documents contained in this web site are presented for information purposes only. This correspondence has been reviewed by an OTRS member and stored in our permission enferkedad.
enfermedad de Degos – English Translation – Word Magic Spanish-English Dictionary
It may more rarely manifest as pericarditis or in other organs such as the lungs, presenting as pleuritis. Genetic counseling A genetic predisposition with an autosomal dominant trait has been suggested.
Wikimedia has received an e-mail confirming that the copyright holder has approved publication under the terms mentioned enfermrdad this page. Diagnostic methods Diagnosis is based primarily on the cutaneous clinical picture that is nearly pathognomonic.
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 neurologic involvementhas also been described. As all patients may potentially develop the systemic, life-threatening variant, an annual follow-up is mandatory. Clinical description MAP onset occurs in enffrmedad aged with skin lesions that appear initially as small erythematous papules, predominantly on the trunk and the upper extremities.
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.
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Thomas Habif authorises the use of the 21 pictures listed here and published on the website dermnet. Prognosis Idiopathic, monosymptomatic, cutaneous presentations are benign, however, systemic manifestations can develop years after the occurrence of skin lesions. I, the copyright holder of this work, drgos publish it under the following license:.
Additional information Further information on this disease Classification s 3 Gene s 0 Clinical signs and symptoms Publications in PubMed Other website s 7. Retrieved from ” https: Health care resources for this disease Expert centres 65 Diagnostic tests 0 Patient organisations 19 Orphan drug s 0.
Systemic lesions may affect the gastrointestinal tract and the central nervous system CNS and are potentially lethal. 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. For all other comments, please send your remarks via contact enfermedadd.
Over several days, the center of the lesions sinks and develops a characteristic morphology: Specialised Social Services Eurordis directory. The histology of early lesions resembles cutaneous lupus erythematosus see this term. Degos disease Injured human backs. Degos disease Date Source http: 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 condition is chronic and lesions persist over years, often throughout life.
Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. From Wikimedia Commons, the free media repository. Many patients have been reported to have defects in blood coagulation. More debos lesions can imitate lichen sclerosus see this term. If you have questions about the archived correspondence, please use the OTRS noticeboard.
In early stages, histology of lesions may reveal a superficial and deep perivascular lymphocytic infiltration with distinct mucin deposition.
Face, scalp, palms of hands and soles of feet are rarely involved. MAP onset occurs in adults aged with skin lesions that appear initially as small erythematous papules, predominantly on the trunk and the upper extremities. A clinical inspection of the skin should be combined with additional examinations including brain magnetic resonance tomography, gastroscopy, colonoscopy, X-ray of the chest and abdominal ultrasound, in order to assess the long-term prognosis.
Permission Reusing this file. Etiology The etiopathogenesis of the disease remains unknown. Summary and related texts.
Systemic manifestations are progressive and may lead to serious complications: More developed lesions show prominent changes in the dermoepidermal junction, with atrophy of the epidermis and an area of sclerosis in the papillary dermis.
If you wish to use this contentenffermedad do not need to request permission as long as you follow any licensing requirements mentioned on this page. Differential diagnosis The histology of early lesions resembles cutaneous lupus erythematosus see this term.
The following other wikis use this file: This page was last edited on 25 Septemberat This work is free and may be used by anyone for any purpose. Summary Enfedmedad Less than cases have been described in the literature. No effective treatment for the systemic manifestations has been established, however, subcutaneous treprostinil has been tested successfully in one case with intestinal and CNS manifestations.
Summary [ edit ] Description Degos-disease. The etiopathogenesis of the disease remains unknown. Only comments written in English can be processed. Other search option s Alphabetical list.