ENFERMEDAD DE DEGOS PDF

English Translation, Synonyms, Definitions and Usage Examples of Spanish Word ‘enfermedad de Degos’. Meanings of “enfermedad de degos” in English Spanish Dictionary: 1 result(s) Meanings of “enfermedad de degos” with other terms in English Spanish. Me llamo Loli tengo 37 aƱos y vivo en Madrid y me diagnoticaron la enfermedad de Degos en despues de que perdiera la vision del ojo.

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Enfermedad de Degos

Only comments written in English can be processed. 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 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 erythematous papules, predominantly on the trunk and the upper extremities. Over several days, the center of the lesions sinks and develops a characteristic morphology: This condition is chronic and lesions persist over years, often throughout life.

Face, scalp, palms of hands and soles of feet are rarely involved. It may more rarely manifest as pericarditis or in other organs such as the lungs, presenting as pleuritis.

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.

The etiopathogenesis of the disease remains unknown. Hypotheses implicating vasculitis, coagulopathy or a primary dysfunction of endothelial cells have been proposed. Many patients have been reported to have defects in blood coagulation.

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Diagnosis is based primarily on the cutaneous clinical picture that dego nearly pathognomonic. In early stages, histology of lesions may reveal a superficial 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 enfermddad, occurs. More developed lesions show prominent changes in the dermoepidermal junction, with atrophy of the epidermis and an area of sclerosis in the papillary dermis.

The histology of early lesions resembles cutaneous lupus erythematosus see this term. More developed lesions can imitate lichen sclerosus see this term.

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. As all patients may potentially develop the systemic, life-threatening variant, an annual follow-up is mandatory.

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. No effective enfermeadd for the systemic manifestations has been established, however, subcutaneous treprostinil has been enfermedaad successfully in one case with intestinal and CNS manifestations.

Idiopathic, monosymptomatic, cutaneous presentations are benign, however, systemic manifestations can develop years after the occurrence of skin lesions. Systemic manifestations dgos progressive and may lead to serious complications: Other search option s Alphabetical list.

Summary and related texts. Check this box if you wish to receive a copy of your message. 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.

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Summary Epidemiology Less than cases have been described in the literature. 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. Etiology The etiopathogenesis of the disease remains unknown. Diagnostic methods Diagnosis is based primarily on the cutaneous clinical picture that is nearly pathognomonic. Differential diagnosis The histology of early lesions resembles cutaneous lupus erythematosus see this term.

Genetic counseling A genetic predisposition with an autosomal dominant trait has been suggested. 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. Prognosis Idiopathic, monosymptomatic, cutaneous presentations are benign, however, systemic manifestations can develop years after the occurrence of skin lesions.

Additional information Further information on this disease Classification s 3 Gene s 0 Clinical signs and symptoms Publications in PubMed Other website s 7.

Orphanet: Enfermedad de Degos

Health care resources for this enfernedad Expert centres 69 Diagnostic tests 0 Patient organisations 21 Orphan drug s 0. Specialised Social Services Eurordis directory. The documents contained in this web site are presented for information purposes only.

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.