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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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. Differential diagnosis The histology of early lesions resembles cutaneous lupus erythematosus see this term.
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.
Enfermedad de Degos
I, the copyright holder of this work, hereby publish it under the following license:. In early stages, histology of lesions may reveal a superficial and deep perivascular lymphocytic infiltration with distinct mucin deposition.
Thomas Habif authorises the use of the 21 pictures listed here and published on the website dermnet. 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.
Systemic manifestations are progressive and may lead to serious complications: The documents contained in this web site are presented for information purposes only. MAP onset occurs in adults aged with skin lesions that appear initially as small erythematous papules, predominantly on the trunk and the upper extremities.
Systemic lesions may affect the gastrointestinal tract and the central nervous system CNS and are potentially lethal. Management and treatment Therapeutic efforts with anticoagulants and compounds that facilitate blood perfusion, such as acetylosalicylic acid, pentoxifylline, dipyridamole, ticlodipine and heparin have achieved enfernedad partial regression of skin lesions in some individual cases. Health care resources for this disease Expert centres 65 Diagnostic tests 0 Patient organisations 19 Orphan drug s 0.
Retrieved from ” https: Specialised Social Services Eurordis directory. Genetic counseling A genetic predisposition with an autosomal dominant trait has been suggested.
Face, scalp, palms of hands ebfermedad soles of feet are rarely involved. It may more rarely manifest as pericarditis or in other organs such as the lungs, presenting as pleuritis.
Degos disease Date Source http: Hypotheses implicating vasculitis, coagulopathy or a primary dysfunction of endothelial cells have been proposed.
Etiology The etiopathogenesis of the disease remains unknown. Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted.
More developed lesions show prominent changes in the dermoepidermal junction, with atrophy of the epidermis and an area of sclerosis in the papillary dermis. For all other comments, please send your remarks via contact us.
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.
Summary [ edit ] Description Degos-disease. Permission Reusing this file. Only comments written in English can be processed.
Other search option s Alphabetical list. 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.
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Additional information Further information on this disease Classification s 3 Gene s 0 Clinical signs and symptoms Publications in PubMed Other website s 7.
The following other wikis use this file: From Wikimedia Commons, the free media repository. 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.
This work is free and may be used by anyone for any purpose. Summary and related texts. Many patients have been reported to have defects in blood coagulation. More developed lesions can imitate lichen sclerosus see this term. Diagnosis is based primarily on the cutaneous clinical picture that is nearly pathognomonic.
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.
Degos disease Injured human backs.