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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Management and treatment Therapeutic efforts with anticoagulants and compounds that facilitate blood perfusion, such dgeos acetylosalicylic acid, pentoxifylline, dipyridamole, ticlodipine and heparin have achieved a partial regression of skin lesions in some individual cases. Wikimedia has received an e-mail confirming that the copyright holder has approved publication under the terms mentioned on this page.
As all patients may potentially develop the systemic, life-threatening variant, an annual follow-up is mandatory.
This condition is chronic and lesions persist over years, often throughout life. Summary and related texts. For all other comments, please send your remarks via contact us.
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The etiopathogenesis of the disease remains unknown. I, the copyright holder of this work, hereby publish it under the following license:. The following ds wikis use this file: The documents contained in this web site are presented for information purposes only. Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted.
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Disease definition Malignant atrophic papulosis MAP is a rare, enfermddad, thrombo-obliterative vasculopathy characterized by papular skin lesions with central porcelain-white atrophy and a surrounding teleangiectatic rim. Etiology The etiopathogenesis of the disease remains unknown. Degos disease Injured human backs. Other search option s Alphabetical list. Therapeutic efforts with anticoagulants and compounds that facilitate blood perfusion, enferemdad as acetylosalicylic acid, pentoxifylline, dipyridamole, ticlodipine and heparin have achieved a partial dsgos of skin lesions in some individual cases.
Diagnostic methods Diagnosis is based primarily on the cutaneous clinical picture that is nearly pathognomonic. The histology of early lesions resembles cutaneous lupus erythematosus see this term.
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 neurologic involvementhas also been described.
Over several days, the center of the lesions sinks and develops a emfermedad morphology: It may more rarely manifest as pericarditis or in other organs such as the lungs, presenting as pleuritis.
Hypotheses implicating vasculitis, coagulopathy dehos a primary dysfunction of endothelial cells have been proposed. More developed lesions show prominent changes in the dermoepidermal junction, with atrophy of the epidermis and an area of sclerosis in the papillary dermis.
Views View Edit History. Summary [ edit ] Description Degos-disease. Thomas Habif authorises the use of the 21 pictures listed here and published on the website dermnet. Differential diagnosis The histology of early lesions resembles cutaneous lupus erythematosus see this term. Specialised Social Services Eurordis directory.
Idiopathic, monosymptomatic, cutaneous presentations are benign, however, systemic manifestations can develop years after the occurrence of endermedad lesions. Health care resources for this disease Expert centres 65 Diagnostic tests 0 Patient organisations 19 Orphan drug s 0. Many patients have been reported to have defects in blood coagulation.
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Systemic lesions may affect the gastrointestinal tract and the central nervous system CNS and are potentially lethal. MAP onset occurs in adults aged with skin lesions that appear initially as small enfermedar papules, predominantly on the trunk and the upper extremities. Prognosis Idiopathic, monosymptomatic, cutaneous presentations are benign, however, systemic degoa can develop years after the occurrence of skin lesions.
In early stages, histology of lesions may reveal a superficial and deep perivascular lymphocytic infiltration with distinct mucin deposition. Clinical description MAP onset occurs in adults aged with skin lesions that appear initially as small erythematous papules, predominantly on the trunk and the upper extremities. Retrieved from ” https: 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.
Genetic counseling A genetic predisposition with an autosomal dominant trait has been suggested. Systemic manifestations are progressive and may lead to serious complications: 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.
Later a wedge-shaped connective tissue necrosis in the deep dermis, due to a thrombotic occlusion of the small arteries and sparse lymphocytes, occurs. Face, scalp, palms of hands and soles of feet are rarely involved.
Summary Epidemiology Less than cases have been described in the literature. This work is free and may be used by anyone for any purpose. Check this box if you wish to receive a copy of your message.