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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Diagnostic methods Diagnosis is based primarily on the cutaneous clinical picture that is nearly pathognomonic.
Prognosis Idiopathic, monosymptomatic, cutaneous presentations are benign, however, systemic manifestations can develop years after the occurrence of skin lesions.
The following other wikis use this file: Systemic lesions may affect the gastrointestinal tract and the central nervous system CNS and are potentially lethal. The histology enfermeda early lesions resembles cutaneous lupus erythematosus see this term.
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.
The documents contained in wnfermedad web site are presented for information purposes only.
Retrieved from ” https: Etiology The etiopathogenesis of the disease remains unknown. Summary and related texts. Over several days, the center of the endermedad sinks and develops a characteristic morphology: Summary Epidemiology Less than cases have been described in the literature.
Idiopathic, monosymptomatic, cutaneous presentations are benign, however, systemic manifestations can develop years after the occurrence of skin lesions.
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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.
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. 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.
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Orphanet: Enfermedad de Degos
This page was last edited on 25 Septemberat In early stages, histology of lesions may reveal a fe and deep perivascular lymphocytic infiltration with distinct mucin deposition. Later a wedge-shaped connective tissue necrosis in the deep dermis, due to a thrombotic occlusion of the small arteries and sparse lymphocytes, occurs.
For all other comments, please send your remarks via contact us. The etiopathogenesis of the disease remains unknown.
enfermedad de Degos – English Translation – Word Magic Spanish-English Dictionary
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. This work is free and may be used by anyone for any purpose.
Specialised Social Services Eurordis directory. Systemic manifestations are progressive and may lead to serious complications: Additional information Further information on this disease Classification s 3 Gene s 0 Ehfermedad signs and symptoms Publications in PubMed Other df s 7. Disease definition Malignant atrophic papulosis MAP is a rare, chronic, thrombo-obliterative vasculopathy characterized by papular skin lesions ebfermedad central porcelain-white atrophy and a surrounding teleangiectatic rim.
Diagnosis is based primarily on the cutaneous clinical picture that is nearly pathognomonic. Degos disease Date Source http: Permission Reusing this file. Only comments written in English can be processed.
MAP onset occurs in adults aged with skin lesions that appear initially as small erythematous papules, predominantly on the trunk and the upper extremities. More developed lesions show prominent changes in the dermoepidermal junction, with atrophy of the epidermis and an area of sclerosis in the papillary dermis. 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 of enfrrmedad lesions in some enfrrmedad cases.
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Face, scalp, drgos of hands and soles of feet are rarely involved. 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.
Hypotheses implicating vasculitis, coagulopathy or a primary dysfunction of endothelial cells have been proposed.
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As all patients may potentially develop the systemic, life-threatening variant, an annual follow-up is mandatory. 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. Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted.
Enfer,edad patients have been reported to have defects in blood coagulation.
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.