ALLMedicine™ Malignant Atrophic Papulosis Center - Clinicaltrials.gov
Dermatology Online Journal; Scheinfeld N
Mar 13th, 2009 - Degos disease is a poorly defined condition that encompasses cutaneous and systemic findings that overlap with a large number of other rheumatologic and coagulation disorders. Comparisons are made herein with 9 conditions that share findings and presentations.
Journal of the European Academy of Dermatology and Venere... Zouboulis CC, Theodoridis A et. al.
May 25th, 2017 - Atrophic papulosis is a rare thrombo-occlusive disease, characterized by the appearance of multiple atrophic porcelain-white skin papules, with a surrounding erythematous rim, which are histologically consisting of wedge-shaped necrosis of the dermis. It consists of two variants: (i) the benign atrophic papulosis (BAP) only involving the skin and (ii) the malignant atrophic papulosis (MAP) also...
Liu CM, Harris RM et. al.
Malignant atrophic papulosis (MAP), or Degos syndrome, is a rare disorder of unknown etiology. It is characterized by a deep subcutaneous vasculopathy resulting in atrophic, porcelain-white papules.
The American Journal of Dermatopathology; Kim E, Motaparthi K
Feb 28th, 2017 - Degos disease manifests as 2 distinct clinical variants, malignant atrophic papulosis and benign atrophic papulosis, which are distinguished by the presence or absence of systemic disease. Both forms feature cutaneous involvement typified by erythematous papules with scar-like centers, and the classic histologic picture is described as wedge-shaped dermal necrosis overlying thrombotic vasculopa...
Dermatology Online Journal; Magro CM, Toledo-Garcia A et. al.
Oct 5th, 2015 - In 2012, a nephrologist reported the development of a multiorgan thrombotic syndromic complex resembling thrombotic thrombocytopenic purpura (TTP) in patients who were abusing long acting oxymorphone hydrochloride; all patients had hemolytic anemia and thrombocytopenia. Herein, we report another case involving a 31-year-old woman who self intravenously administered dissolved oral oxymorphone re...
Pediatric Dermatology; Gutiérrez-Pascual M, Hernández-Martín A et. al.
Dec 7th, 2010 - Malignant atrophic papulosis, or Degos' disease, is a severe systemic vasculopathy extremely rare in children. The skin, gastrointestinal tract, and central nervous system are most frequently affected. We report a 5-year-old girl with malignant atrophic papulosis who presented with widespread skin lesions from early infancy and severe visual and neurological impairment.
Surgery Today; Kim DW, Kang SB et. al.
Aug 29th, 2008 - Degos' disease, otherwise known as "malignant atrophic papulosis," is a rare condition characterized by typical cutaneous lesions. Its involvement of the gastrointestinal (GI) tract is usually associated with a poor prognosis. We report a case of Degos' disease with GI involvement, which ultimately caused peritonitis, sepsis, and death, despite all treatment measures. A 59-year old woman was ad...
Gastroenterology Yamaguchi N, Isomoto H et. al.
Oct 14th, 2008 - Clinical challenges and images in GI. Image 2: Malignant atrophic papulosis (Degos' disease) with systemic manifestations.|2008|Yamaguchi N,Isomoto H,Nakayama T,|complications,diagnosis,surgery,complications,diagnosis,surgery,methods,complications,diagnosis,diagnosis,etiology,surgery,pathology,
BMC Gastroenterology; Toledo AE, Shapiro LS et. al.
Nov 3rd, 2015 - The malignant form of atrophic papulosis (Köhlmeier-Degos disease) is a rare thrombo-occlusive vasculopathy that can affect multiple organ systems. Patients typically present with distinctive skin lesions reflective of vascular drop out. The small bowel is the most common internal organ involved, resulting in considerable morbidity and mortality attributable to ischemic microperforations. Deter...
Rheumatic Diseases Clinics of North America; Miloslavsky EM, Stone JH et. al.
Nov 17th, 2014 - The need to distinguish true primary systemic vasculitis from its multiple potential mimickers is one of the most challenging diagnostic conundrums in clinical medicine. This article reviews 9 challenging vasculitis mimickers: fibromuscular dysplasia, calciphylaxis, segmental arterial mediolysis, antiphospholipid syndrome, hypereosinophilic syndrome, lymphomatoid granulomatosis, malignant atrop...
Journal of the Neurological Sciences; Pati S, Muley SA et. al.
Nov 2nd, 2010 - Degos' disease or malignant atrophic papulosis is a rare disseminated occlusive vasculopathy affecting the skin, gastrointestinal tract, central nervous system, and less often other organ systems. The exact etiology of this vasculopathy has not been established. Infections, autoimmune disease and coagulation defects have been proposed as underlying pathogenic mechanisms, but none have been conf...
Orphanet Journal of Rare Diseases; Shapiro LS, Toledo-Garcia AE et. al.
Apr 5th, 2013 - 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 disease prog...
Clinical and Experimental Dermatology; Guhl G, Diaz-Ley B et. al.
Dec 1st, 2008 - Wegener's granulomatosis (WG) is a multisystemic vasculitis, with skin involvement in 14% of cases and with palpable purpura, subcutaneous nodules and necrotic papules as the common features.(1) We present a patient diagnosed with WG who had multiple whitish papules similar to those of malignant atrophic papulosis (Degos' disease), which appeared during a flare of his disease. Lesions of malign...
Journal of the American Academy of Dermatology; Meephansan J, Komine M et. al.
Sep 6th, 2012 - Degos disease or malignant atrophic papulosis is a rare occlusive vasculopathic disease characterized by pathognomonic cutaneous lesions and frequently fatal systemic involvement. The etiology of malignant atrophic papulosis remains unclear, and there is currently no effective treatment for malignant atrophic papulosis. Several chemokines can potentiate and expand the platelet response to incre...
The Journal of International Medical Research; Zheng XY, Huang DY et. al.
Sep 7th, 2010 - Malignant atrophic papulosis (MAP) is a rare disease with an extremely grim prognosis, death being due to gastrointestinal perforation and neurological disorders. We report a severe case of MAP in a 37-year old woman. The patient had three emergent laparotomies in 3 months for recurrent acute peritonitis due to omental thrombosis resulting in necrosis, multiple intestinal and mesenteric lesions...
Orphanet Journal of Rare Diseases; Theodoridis A, Makrantonaki E et. al.
Jan 15th, 2013 - 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. Less than 200 cases have been described in the literature. The first manifestation of MAP usually occurs between ...
The Journal of Dermatology; Hu P, Mao Z et. al.
Mar 8th, 2018 - Malignant atrophic papulosis (MAP) is a rare type of obliterating vasculopathy that can present as pure cutaneous lesions or a systemic entity affecting multiple organs. Systemic disease, such as gastrointestinal or central nervous system involvement, may predispose the patients to poorer or even fatal outcomes. We present a 30-year-old female patient with systemic manifestation of MAP 10 days ...
Orphanet Journal of Rare Diseases; Magro CM, Wang X et. al.
Nov 27th, 2013 - Degos disease is a frequently fatal and incurable occlusive vasculopathy most commonly affecting the skin, gastrointestinal tract and brain. Vascular C5b-9 deposition and a type I interferon (IFN) rich microenvironment are held to be pathogenetically important in the evolution of the vascular changes. We recently discovered the use of eculizumab as a salvage drug in the treatment of near fatal ...
Clinical and Experimental Dermatology; Wallace MP, Thomas JM et. al.
Jul 11th, 2017 - Systemic lupus erythematosus (SLE) is an autoimmune disease. Its incidence in the UK is approximately 1 per 10 000. Cutaneous involvement, encompassing acute, subacute and chronic disease, occurs in over two-thirds of cases, and can often be the first clue to diagnosis. We describe a highly unusual case of SLE occurring after prodromal idiopathic thrombocytopenic purpura (ITP) and presenting wi...
International Journal of Dermatology; Su Z, Lu Y et. al.
Jun 21st, 2013 - Central nervous system involvement in systemic malignant atrophic papulosis (Degos disease): a case report.|2013|Su Z,Lu Y,Ge Y,Jiang J,Jia Z,|diagnosis,etiology,complications,diagnosis,