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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The following other wikis use this file: Only comments written in English can be processed. Diagnosis is based primarily on the cutaneous clinical picture that is nearly pathognomonic.
Etiology The etiopathogenesis of the disease remains unknown.
Retrieved from ” https: 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. 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 skin lesions in some individual cases.
Summary and related texts.
enfermedac Over several days, the center of the degis sinks and develops a characteristic morphology: The etiopathogenesis of the disease remains unknown. If you have questions about the archived correspondence, please use the OTRS noticeboard. 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.
Face, scalp, palms of hands and soles of feet are rarely involved. Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted.
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.
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. Check this box if you wish to receive degs copy of your message. In early stages, histology of lesions may reveal a superficial and deep perivascular lymphocytic infiltration with distinct mucin deposition.
MAP onset occurs in adults aged with skin lesions that appear initially as small erythematous papules, predominantly on the trunk and the upper degow.
Summary [ edit ] Description Degos-disease. Specialised Social Services Eurordis directory. 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.
More developed lesions can imitate lichen sclerosus see this term.
Many patients have been reported to have defects in blood coagulation. The documents contained in this web site are presented for information purposes only.
Later a wedge-shaped connective tissue necrosis in the deep dermis, due to a thrombotic occlusion of the small arteries and sparse lymphocytes, occurs. The histology of early lesions resembles cutaneous lupus erythematosus see this term. This page was last edited on 25 Septemberat 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, hereby publish it under the following license:. Diagnostic methods Diagnosis is based primarily on the cutaneous clinical picture that is nearly pathognomonic.
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. Thomas Habif authorises the use of the 21 pictures listed here and published on the website dermnet. For all other comments, please send your remarks via contact us.
Degos disease Injured human backs.