Köhlmeier-Degos Disease (malignant atrophic papulosis) and Neurologic Involvement
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چکیده
منابع مشابه
Köhlmeier-Degos Disease (malignant atrophic papulosis) and neurologic involvement.
Dr. Felipe Slaviero – Serviço de Neurologia e Neurocirurgia Rua Teixeira Soares 640 99010-901 Passo Fundo RS Brasil. E-mail: [email protected] The malignant atrophic papulosis (MAP) was first described by Köhlmeier in 1941 and recognized as a specific entity by Degos in 1942. This rare disorder is an obstructive vasculopathy of unknown origin, characterized by vascular lesions of the s...
متن کاملMalignant atrophic papulosis (Köhlmeier-Degos disease) - A review
UNLABELLED DEFINITION OF THE DISEASE: Malignant atrophic papulosis (MAP), described independently by Köhlmeier and Degos et al., is a rare, chronic, thrombo-obliterative vasculopathy characterized by papular skin lesions with central porcelain-white atrophy and surrounding teleangiectatic rim. EPIDEMIOLOGY Less than 200 cases have been described in the literature. The first manifestation of M...
متن کاملRenal involvement in Malignant Atrophic Papulosis (Degos Disease)*
To Editor: We read with interest the article of Dr. Lima on Malignant Atrophic Papulosis (MAP) or Degos Disease (DD) 1. This rare disease is an occlusive vasculopathy characterized by skin lesions and systemic involvement. DD is a potentially life-threatening disease, mainly the involvement of inner organs (bowel perforation and peritonitis, massive cerebral haemorrhage, thrombosis of the cereb...
متن کاملEffective treatment of malignant atrophic papulosis (Köhlmeier-Degos disease) with treprostinil – early experience
BACKGROUND Malignant atrophic papulosis (Köhlmeier-Degos disease; MAP) is an uncommon endotheliopathy with pathological findings similar to the vascular lesions of systemic sclerosis. These two disorders can overlap. When associated with visceral lesions, MAP has been considered almost universally and rapidly fatal. A recent report described dramatic response to treatment with eculizumab, but d...
متن کاملDegos disease – malignant atrophic papulosis or cutaneointestinal lethal syndrome: rarity of the disease
BACKGROUND Degos disease is a very rare syndrome with a rare type of multisystem vasculopathy of unknown cause that affects the skin, gastrointestinal tract, and central nervous system. Other organs such as the kidneys, lungs, pleura, liver, heart, and eyes, can also be involved. OBJECTIVE To highlight the incidence of Degos disease with regard to age and sex, discuss the necessity of its acc...
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ژورنال
عنوان ژورنال: Arquivos de Neuro-Psiquiatria
سال: 2009
ISSN: 0004-282X
DOI: 10.1590/s0004-282x2009000400022