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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Thomas Habif authorises the use of the 21 pictures listed here and published on the website dermnet. No effective treatment for the systemic manifestations has been established, however, subcutaneous treprostinil has been tested successfully in one case devos intestinal and CNS manifestations.
The histology of early lesions resembles cutaneous lupus erythematosus see this term.
Systemic lesions may affect the gastrointestinal tract and the central nervous system CNS and are potentially lethal. The etiopathogenesis of the disease remains unknown.
Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. Retrieved from ” https: 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.
Views View Edit History. Summary [ edit ] Description Degos-disease. 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.
Specialised Social Services Eurordis directory. Many patients have been reported to have defects in blood coagulation. The material is in no way intended to replace enfermsdad medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment. Face, scalp, palms of hands and soles of feet are rarely involved.
Orphanet: Enfermedad de Degos
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. Prognosis Idiopathic, monosymptomatic, cutaneous presentations are benign, however, systemic manifestations can develop years after the occurrence of skin lesions. 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.
If you wish to use this contentyou do not need to request permission as long as enfdrmedad follow any licensing requirements mentioned on this page. More developed lesions can imitate lichen sclerosus see this term.
Degos disease Injured human backs. As all patients may potentially develop the systemic, life-threatening variant, an annual follow-up is enfermedav.
Enfermedad de Degos
Hypotheses implicating vasculitis, coagulopathy or a primary dysfunction of endothelial cells have been proposed. Management and treatment Therapeutic efforts with anticoagulants and compounds that facilitate blood perfusion, such snfermedad acetylosalicylic acid, pentoxifylline, dipyridamole, ticlodipine and heparin have achieved a partial regression of skin lesions in some individual cases.
In early stages, histology of lesions may reveal a superficial and deep perivascular lymphocytic infiltration with distinct mucin deposition. Check this box if you wish to receive a copy of your message. More developed lesions show prominent changes in the dermoepidermal junction, with atrophy of the epidermis and an area of sclerosis in the papillary dermis. Summary and related texts. Other search option s Alphabetical list.
Permission Reusing this file. This page was last edited on 25 Septemberat 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. MAP onset occurs in adults aged with skin lesions that appear initially as small erythematous papules, predominantly on the trunk and the upper extremities.
Additional information Further information on this disease Classification cegos 3 Gene s 0 Clinical signs and symptoms Publications in PubMed Other website s 7. This condition is chronic and lesions persist over years, often throughout life. Diagnosis is based primarily on the cutaneous clinical picture that is nearly pathognomonic.
For all other enfermedd, please send your remarks via contact us. This correspondence has been reviewed by an OTRS member and stored in our permission archive. Degos disease Date Source http: