We describe an 18 year old male who first presented with cutaneous atrophic papulosis but became critically ill from small bowel microperforations. He had an almost immediate and dramatic response to treatment. [ncbi.nlm.nih.gov]

• Pain

  She also was experiencing frequent abdominal pain. Laparoscopy was performed but no serosal lesion suggestive of MAP was seen and her abdominal pain spontaneously abated. [doi.org]

  We hypothesize that these lesions start as painful red nodules and may represent part of the disease spectrum in the evolution of MAP. [ncbi.nlm.nih.gov]

• Asymptomatic

  A 29-year-old Japanese man with malignant atrophic papulosis had a 1-year history of scattered, asymptomatic, whitish or skin-colored papules, as well as erythematous papules with central atrophy showing a porcelain-like appearance on the trunk and extremities [ncbi.nlm.nih.gov]

  Number of Print Pages: 2 Number of Figures: 0 Number of Tables: 0 ISSN: 1018-8665 (Print) eISSN: 1421-9832 (Online) For additional information: https://www.karger.com/DRM Abstract We describe a 34-year-old woman presenting with a 1 -year history of asymptomatic [karger.com]

  Characteristic skin papular lesions are described as pink, circular in shape, bumpy, asymptomatic, diffuse with a telangiectatic border followed by umbilication and a porcelain white center appearance. [accessanesthesiology.mhmedical.com]

  A 44-year-old man had episodes of generalized asymptomatic eruptions of two years duration, and also an abdominal crisis which lasted for three days. [ci.nii.ac.jp]

• Weight Loss

  When the GI tract is involved, it can result in abdominal pain, diarrhea, weight loss, and even intestinal perforation Degos Disease is a complex and progressive condition that has no cure. [dovemed.com]

  Some affected individuals may experience weakness, fatigue and weight loss from malabsorption. The intestinal lesions can tear or rupture (perforate) causing the contents of the intestines to leak into the abdominal cavity. [rarediseases.org]

  0012378 Gastrointestinal hemorrhage Gastrointestinal bleeding 0002239 Gastrointestinal infarctions Death of digestive organ tissue due to poor blood supply 0005244 Intestinal perforation 0031368 Muscle flaccidity 0010547 Nausea and vomiting 0002017 Weight [rarediseases.info.nih.gov]

  He then began to complain of fatigue and experienced weight loss of 15 kg over a period of 2 months. He was admitted due to abdominal pain. [medicaljournals.se]

  Weight loss at an average of 6–10 kg per month is common. A repeat laparotomy is frequently performed because of intestinal occlusion by adherences or acute peritonitis. [dovepress.com]

• Falling

  Zimbabwe : Bulawayo, Harare, Mutare, Victoria Falls, etc. [maria-online.com]

  Degos disease falls under the alternative appellation of malignant atrophic papulosis based on the fatal outcome that frequently characterizes its clinical course. 1–7 Morphologic features of Degos disease include a pauci-inflammatory thrombotic microangiopathy [doi.org]

• Pathologist

  As physicians, radiologists and even pathologists have difficulty in diagnosing this condition that appears under multiple guises the information contained here should be invaluable. Alexander R. Margulis, M. D. [books.google.com]

  Pathologist reported that histopathological features in specimens from skin and jejunal lesions are similar and show dense dermal fibrosis and submucosal collagen deposition, respectively. [e-ijd.org]

• Abdominal Pain

  She also was experiencing frequent abdominal pain. Laparoscopy was performed but no serosal lesion suggestive of MAP was seen and her abdominal pain spontaneously abated. [doi.org]

  A 59-year old woman was admitted to our hospital with acute generalized abdominal pain. [ncbi.nlm.nih.gov]

  We report the case of a 60-year-old man who presented with sudden visual loss, a history of postprandial abdominal pain, malabsorption, and skin lesions typical of systemic Degos’ disease. [tandfonline.com]

• Vomiting

  The systemic variant, in addition to the skin papules, is characterized by gastrointestinal manifestations including abdominal pain and distention, nausea, vomiting, diarrhea, intestinal infarction, and intestinal perforation. [linkedlifedata.com]

  The usual symptoms are sudden onset of abdominal pain or gastrointestinal bleeding (vomiting blood or passing blood with the bowel motion). [dermnetnz.org]

  Diarrhea, constipation Weight loss Fatigue Ischemic colitis, which is injury to the colon due to inadequate blood supply Blood in vomit Blood in diarrhea 1 in 5 individuals show neurological signs and symptoms including headache and dizziness Chest pain [dovemed.com]

  […] arteriopathy ● Progressive, small- and medium- size arterial occluding disease ● Leads to tissue infarction ● It initially involves the skin (systemic lesions may rarely precede skin lesions) Classification 6 7 Gastrointestinal tract: abdominal pain, nausea, vomiting [slideplayer.com]

  When lesions form in the small intestines, they may cause abdominal pain, cramping, nausea, vomiting, diarrhea, bloating, constipation, and the passing of blood with bowel movements or vomiting up of blood. [rarediseases.org]

• Nausea

  The systemic variant, in addition to the skin papules, is characterized by gastrointestinal manifestations including abdominal pain and distention, nausea, vomiting, diarrhea, intestinal infarction, and intestinal perforation. [linkedlifedata.com]

  […] arteriopathy ● Progressive, small- and medium- size arterial occluding disease ● Leads to tissue infarction ● It initially involves the skin (systemic lesions may rarely precede skin lesions) Classification 6 7 Gastrointestinal tract: abdominal pain, nausea [slideplayer.com]

  In 50% of cases GIT is affected in the form of nausea, vomiting, bowel movement & bleeding while in 20 % of cases C.N.S. is affected in the form of headache, dizziness and paraplegia. [edoj.org.eg]

  A 48-year-old Caucasian man presented to the emergency department with 5-day history of generalized abdominal pain, nausea, and vomiting which deteriorated and finally developed bloody diarrhea. [e-ijd.org]

  […] either before or after development of systemic signs and symptoms In case of systemic involvement, it may result in the following additional conditions (apart from skin manifestations): Involvement of the digestive (GI) tract causing: Abdominal pain Nausea [dovemed.com]

• Diarrhea

  The systemic variant, in addition to the skin papules, is characterized by gastrointestinal manifestations including abdominal pain and distention, nausea, vomiting, diarrhea, intestinal infarction, and intestinal perforation. [linkedlifedata.com]

  When the GI tract is involved, it can result in abdominal pain, diarrhea, weight loss, and even intestinal perforation Degos Disease is a complex and progressive condition that has no cure. [dovemed.com]

  In some patients, history and/or review of systems will reveal signs indicative of extra- cutaneous involvement: abdominal pain, diarrhea, melena, nausea, blurred vision, hemiparesis, paresthesia or any other sign indicative of an ischemic event. [dermaamin.com]

  When lesions form in the small intestines, they may cause abdominal pain, cramping, nausea, vomiting, diarrhea, bloating, constipation, and the passing of blood with bowel movements or vomiting up of blood. [rarediseases.org]

  Most malignant cases involve problems of the gastrointestinal tract leading to small intestine lesions, abdominal pain, diarrhea, and bowel perforation. [en.wikipedia.org]

• Chronic Abdominal Pain

  Jalil et al [17] described a case in a 2-year-old child who presented with chronic abdominal pain of uncertain origin. The benign cutaneous variant of Degos disease can occur in adults. [emedicine.com]

• Vascular Disease

  During the past three decades, five patients with a collagen vascular disease have been reported to have MAP-like lesions as a manifestation of their underlying illness. [ncbi.nlm.nih.gov]

  […] title=Category:Degos_disease&oldid=307389478 " Categorie : Diseases and disorders of the skin Vascular diseases Categoria nascosta: Uses of Wikidata Infobox [commons.wikimedia.org]

  Kanwar, Degos Disease with Dermatomyositis-like Phenomenon: A Diagnostic Dilemma and a Therapeutic Challenge, Journal of Cutaneous Medicine and Surgery, 15, 3, (162), (2011) ., Cutaneous Vascular Diseases, Andrews' Diseases of the Skin, 10.1016/B978-1 [doi.org]

  Degos Disease (Malignant Atrophic Papulosis, Köhlmeier-Degos Disease) Degos Disease is an ultra-rare vascular disease presenting as skin lesions (porcelain plaques) with an erythematous, telangiectatic rim. [steffens-scleroderma.org]

• Blurred Vision

  In some patients, history and/or review of systems will reveal signs indicative of extra- cutaneous involvement: abdominal pain, diarrhea, melena, nausea, blurred vision, hemiparesis, paresthesia or any other sign indicative of an ischemic event. [dermaamin.com]

• Skin Lesion

  The disease began in infancy with rare recurring skin lesions; the child died at the age of 7, after progressive neurologic deterioration. Diagnosis was suspected clinically and confirmed by biopsy of a typical skin lesion. [scholars.northwestern.edu]

  lesions from a clinical and histological standpoint. [unboundmedicine.com]

  The vascular lesion is localized to the intimal layer with proliferation of spindle-shaped cells indentical to the gastrointestinal lesion of malignant atrophic papulosis (Degos' disease). The patient had no skin biopsy, or history of skin lesions. [ncbi.nlm.nih.gov]

• Papule

  The skin disease shows erythematous papules with characteristic porcelain-white centers and elevated borders. [medical-dictionary.thefreedictionary.com]

  It is characterized by a deep subcutaneous vasculopathy resulting in atrophic, porcelain-white papules. [ncbi.nlm.nih.gov]

  Peristomal atrophic papules. Degos' disease (malignant atrophic papulosis). Archives of Dermatology, 128 (2). DOI: 10.1001/archderm.128.2.256 Bulengo-Ransby, SM, Burns, MK, Taylor, WB, Ellis, CN & Voorhees, JJ 1992, ' Peristomal atrophic papules. [experts.umich.edu]

• Eruptions

  We describe a 33-year-old man who presented with a widespread skin eruption consistent with malignant atrophic papulosis. [ncbi.nlm.nih.gov]

  Rash, Myalgia, and Weakness Posted by • June 13th, 2014 In the latest Case Record of the Massachusetts General Hospital, a 32-year-old man was admitted to this hospital because of a violaceous eruption that was followed by muscle pain and weakness, leading [blogs.nejm.org]

• Erythema

  For these reasons, we consider Degos' disease to be a distinctive pattern of disease, rather than a specific disease per se, just as are erythema multiforme, erythema nodosum, leukocytoclastic vasculitis, Sweet's syndrome, and pyoderma gangrenosum, to [dx.doi.org]

  Prior to his presentation with acute abdomen he had upper and lower endoscopy showing areas of nonspecific patchy erythema. At laparotomy, innumerable characteristic lesions with central pearly hue and erythematous border were seen. [ncbi.nlm.nih.gov]

  Numerous atrophic white or yellow-white lesions surrounded by a telangiectatic rim of erythema were widely scattered over the trunk and extremities. [ci.nii.ac.jp]

  Characterized by numerous typical porcelain-white, atrophic papules, with a rim of rosy erythema and/or telangiectases. Pathology shows a wedge-shaped area of dermal necrosis with edema and mucin deposition. [dermaamin.com]

  Erythema multiforme in a patient with acute human parvovirus B19 infection. J Am Acad Dermatol 1989; 20 (5 Pt 1): 849-50. [scielo.conicyt.cl]

• Skin Rash

  The patient had had a symptomless, pathognomonic skin rash for 6 months and thereafter also multiple intestinal infarctions which caused perforations, peritonitis, and finally death. [ncbi.nlm.nih.gov]

  More or less, the first manifestation of DD is skin rash and in about 15% of patients, disease remains limited to skin (benign form) whereas in others progresses to systemic involvement (malignant form). [e-ijd.org]

  Degos' disease: acute abdomen with skin rash. Indian J Gastroenterol. 2006 Jul-Aug. 25(4):187. [Medline]. Kim DW, Kang SB, Lee KH, Choe GY, Park SY, Nicholay M. [emedicine.com]

• Stroke

  Roach chaired the AHA panel that produced the consensus guidelines on childhood stroke and Drs. Lo and Heyer have published extensively on stroke and related topics [books.google.com]

  Blecic and Julien Bogousslavsky, Chapter 8 Unusual Causes of Stroke, Prevention and Treatment of Ischemic Stroke, 10.1016/S1877-3419(09)70083-2, (139-174), (2004). Elizabeth Ball, Amy Newburger and A. [doi.org]

Treprostinil may offer a second effective treatment approach to individuals with MAP or "rescue therapy" to those in whom eculizumab treatment has failed to maintain suppression of disease activity. [ncbi.nlm.nih.gov]

Conclusions Treprostinil may offer a second effective treatment approach to individuals with MAP or “rescue therapy” to those in whom eculizumab treatment has failed to maintain suppression of disease activity. [doi.org]

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. [ncbi.nlm.nih.gov]

Prognosis Due to the markedly different prognosis between the apparently idiopathic cutaneous disease and MAP with systemic involvement, the first variant - in contrast to the latter "malignant" one - has been termed "benign atrophic papulosis" by some [doi.org]

The first chapter stresses the etiology and pathophysiology of childhood stroke as the basis for evaluations and treatment. [books.google.com]

The etiology and pathophysiology are unknown.,NCI: A rare disorder of unknown etiology. It may present as a cutaneous disorder with a benign clinical course or a systemic disorder with a grim prognosis. [linkedlifedata.com]

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. [ncbi.nlm.nih.gov]

The etiology and pathophysiology are unknown. [purl.bioontology.org]

▪ EPIDEMIOLOGY Classic Degos disease is rare, with less than 200 reported cases. It almost always occurs in Caucasians, but cases have been observed in African American patients and in Japan. [dermaamin.com]

This finding supports the theory that Degos disease is perhaps, in part, an endothelial disease. [13] Epidemiology Frequency Degos disease is rare. About 200 cases have been reported in the world literature. [emedicine.com]

Asymmetric periflexural exanthem of childhood: a clinical, pathologic, and epidemiologic prospective study. Arch Dermatol 1999; 135 (7): 799-803. [scielo.conicyt.cl]

Etiology / Pathophysiology 14 ● Familial cases: Autosomal dominant mode of inheritance (?) ● Are familial cases more benign than sporadic ones ? 32 cases in 10 families: 4 cases with malignant course (12.5%) ! [slideplayer.com]

Gain fresh perspectives and up-to-date insights from the world’s leading authorities on the pathophysiology, diagnosis, and management of stroke. [books.google.com]

Our review of the reported intrathoracic involvement in the more than 60 reported cases of Degos' disease suggests that pleuritis and pericarditis are manifestations of the underlying pathophysiologic process. [ncbi.nlm.nih.gov]

The etiology and pathophysiology are unknown. [purl.bioontology.org]

Prevention of coronary heart disease with pravastatin in men with hypercholesterolemia. ‏ [books.google.com]

Despite this very intensive anticoagulant and anti-platelet therapy, the treatment had no effect on the skin lesions and could not prevent systemic involvement. [ncbi.nlm.nih.gov]

Blecic and Julien Bogousslavsky, Chapter 8 Unusual Causes of Stroke, Prevention and Treatment of Ischemic Stroke, 10.1016/S1877-3419(09)70083-2, (139-174), (2004). Elizabeth Ball, Amy Newburger and A. [doi.org]

In most cases of familial inheritance, the benign variant of the disease has been present.Due to the lack of knowledge of the pathomechanism for this condition prevention strategies are not known. [en.wikipedia.org]