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1. EMedicine - Malignant Atrophic Papulosis : Article By Hemant Pande, MD
malignant atrophic papulosis Kohlmeier described a case of malignant atrophicpapulosis (MAP) as a form of thromboangiitis obliterans in 1941.
http://www.emedicine.com/med/topic2943.htm
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Malignant Atrophic Papulosis
Last Updated: February 15, 2002 Rate this Article Email to a Colleague Synonyms and related keywords: MAP, Kohlmeier-Degos syndrome, Degos disease, vascular occlusive disorders, thromboangiitis obliterans AUTHOR INFORMATION Section 1 of 10 Author Information Introduction Clinical Differentials ... Bibliography
Author: Hemant Pande, MD , Former Fellow, Department of Gastroenterology, Johns Hopkins Bayview Medical Center Coauthor(s): Lawrence Cheskin, MD , Chief, Associate Professor, Department of Medicine, Division of Gastroenterology, Bayview Medical Center, Johns Hopkins University School of Medicine Brian Lacy, MD , Medical Director of Motility Center, Assistant Professor, Department of Internal Medicine, Division of Gastroenterology, Bayview Medical Center, Johns Hopkins University

2. Degos Disease - 1
Degos' Disease malignant atrophic papulosis. Return to Image Index page.
http://tray.dermatology.uiowa.edu/Degos01.htm
Dept. of Dermatology - University of Iowa College of Medicine
Degos' Disease - Malignant Atrophic Papulosis
Return to Image Index page. Return to Image Index page.
Return to Dermatology's Home page.
September, 1995

3. Arch Dermatol -- Page Not Found
DIAGNOSIS malignant atrophic papulosis (Degos disease). HISTOPATHOLOGIC FINDINGSThe epidermis appears thin. 3. Degos R. malignant atrophic papulosis.
http://archderm.ama-assn.org/issues/v134n2/ffull/dof0298-4b.html
Select Journal or Resource JAMA Archives of Dermatology Facial Plastic Surgery Family Medicine (1992-2000) General Psychiatry Internal Medicine Neurology Ophthalmology Surgery MSJAMA Science News Updates Meetings Peer Review Congress
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4. Degos Disease In A Patient With Acquired Immunodeficiency Syndrome.
Abstract malignant atrophic papulosis is a rare disorder characterized by pathognomonic cutaneous lesions that consist
http://www.aegis.com/pubs/aidsline/1998/aug/M9881102.html
Important note: Information in this article was accurate in 1998. The state of the art may have changed since the publication date.
Degos disease in a patient with acquired immunodeficiency syndrome. J Am Acad Dermatol. 1998 May;38(5 Pt 2):852-6. Unique Identifier : AIDSLINE MED/98252499
Requena L; Farina C; Barat A; Department of Dermatology, Fundacion Jimenez Diaz, Universidad; Autonoma, Madrid, Spain. Abstract: Malignant atrophic papulosis is a rare disorder characterized by pathognomonic cutaneous lesions that consist of infarctive thrombosis. Visceral involvement often occurs; the gastrointestinal tract and the central nervous system are most frequently involved. Malignant atrophic papulosis has not been previously described in an AIDS patient. We describe a 58-year-old homosexual man with AIDS who developed typical cutaneous lesions of malignant atrophic papulosis. No visceral involvement has been detected in 2 years. Keywords: *Acquired Immunodeficiency Syndrome/COMPLICATIONS *Skin Diseases, Papulosquamous/COMPLICATIONS *Skin Diseases, Vascular/COMPLICATIONS
National Library of Medicine
. Reproduced under license with the National Library of Medicine, Bethesda, MD.

5. DermIS / Main Menu / DOIA / Malignant Atrophic Papulosis / Images
malignant atrophic papulosis / images
http://www.dermis.net/bilddb/diagnose/englisch/i447800.htm
HOME DOIA PeDOIA Skincancer ... DOIA Malignant Atrophic Papulosis images
images for the diagnosis 'Malignant Atrophic Papulosis'
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6. Heart Disease - Ludwig Angina - Webmd - Malignant Atrophic Papulosis
Welcome to the WebMD Heart disease Center, learn about symptoms, treatments and more for malignant atrophic papulosis.
http://redirect-west.inktomi.com/click?u=http://ads.247wsr.com/6103-5920546-1-8-

7. Malignant Atrophic Papulosis - General Practice Notebook
Notebook. malignant atrophic papulosis. Dego's disease is a rare, lethalcondition affecting the skin, gut and nervous system. Presentation
http://www.gpnotebook.co.uk/cache/60424212.htm
malignant atrophic papulosis Dego's disease is a rare, lethal condition affecting the skin, gut and nervous system. Presentation:
  • skin lesions are pathognomonic - circular, porcelain- white, with a depressed centre (4 to 8mm diameter), and slightly elevated erythematous margin. arterial lesion - endothelial swelling causing luminal stenosis. There may be fibrinoid necrosis of the wall of the lumen.
Death is generally as a result of intestinal perforation.
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8. EMedicine - Malignant Atrophic Papulosis : Article Excerpt By: Hemant Pande, MD
malignant atrophic papulosis Kohlmeier described a case of malignant atrophic papulosis (MAP) as a form of thromboangiitis obliterans in 1941. It was recognized as a distinct clinical entity by Degos in 1942, hence the name. Excerpt from malignant atrophic papulosis. Synonyms, Key Words, and Related Terms MAP, Kohlmeier-Degos syndrome, Degos
http://www.emedicine.com/med/byname/malignant-atrophic-papulosis.htm
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Excerpt from Malignant Atrophic Papulosis
Synonyms, Key Words, and Related Terms: MAP, Kohlmeier-Degos syndrome, Degos disease, vascular occlusive disorders, thromboangiitis obliterans
Please click here to view the full topic text: Malignant Atrophic Papulosis
Background: Kohlmeier described a case of malignant atrophic papulosis (MAP) as a form of thromboangiitis obliterans in 1941. It was recognized as a distinct clinical entity by Degos in 1942, hence the name. Pathophysiology: MAP is a multisystem disorder involving the small-caliber blood vessels. The disease is characterized by narrowing and occlusion of the lumen by intimal proliferation and thrombosis, which leads to ischemia and infarction in the involved organ systems. MAP is different from other vasculitides in that inflammation is not a prominent component of the disease. MAP may involve the gastrointestinal and genitourinary tracts, central and peripheral nervous systems, skin, heart, lungs, eyes, pancreas, adrenals, and kidneys. The disease involves the skin alone in 37% of cases. The gastrointestinal tract is involved in about 50% of cases. Frequency:
  • Internationally: This is a rare disease, with fewer than 150 cases reported. Most of the cases are sporadic, although members of the same family reportedly have been affected.

9. Www.GPnotebook.co.uk (letter M , Terms 81 To 100)
male puberty male sterilisation malformation (congenital) Malgaigne's luxationmaligant disease in childhood malignant atrophic papulosis malignant central
http://www.gpnotebook.co.uk/list.cfm?ID=m&start=81

10. Degos Disease
An in depth look at degos disease including prognosis and treatment.Category Health Conditions and Diseases Rare Disorders Degos...... The skin biopsy places the pathologist at the center of the diagnostic evaluation.SYNONYMS, malignant atrophic papulosis KöhlmeierDegos' disease.
http://www.thedoctorsdoctor.com/diseases/degos_disease.htm
Background This rare disease is important because a skin biopsy may be the first clue to the diagnosis. Patients classically develop multiple skin papules that evolve to have a dimpled appearance with a porcelain white center. This is the clue to the underlying disease which can affect the gastrointestinal tract and central nervous system and may lead to death. The skin biopsy places the pathologist at the center of the diagnostic evaluation. SYNONYMS Malignant Atrophic Papulosis
Köhlmeier-Degos' disease INCIDENCE Rare DISEASE ASSOCIATIONS CHARACTERIZATION Familial cases Rare PATHOGENESIS CHARACTERIZATION Unknown-has been considered a vasculitis, mucinosis, or thrombotic disorder No circulating immune complexes, anti-endothelial cell antibodies, or anticardiolipin antibodies identified Endovasculitis Some consider a primary endothelial cell defect with secondary thrombosis leading to infarctive changes LABORATORY/RADIOLOGIC/OTHER TESTS CHARACTERIZATION Impaired fibrinolytic activity Alterations in platelet function GROSS APPEARANCE/CLINICAL VARIANTS CHARACTERIZATION Classic Skin Lesions Crops of papules ranging from 0.5-1 cm which develop an umbilication with a porcelain white center with a telangiectatic ring

11. Malignant Atrophic Papulosis (Degos Disease)
Selected medical images. OMIM. malignant atrophic papulosis (Degos disease)
http://david.hcuge.ch/gfmer/Genetic_diseases/Malignant_atrophic_papulosis/Malign
Geneva Foundation for Medical Education and Research Home About Search Medical education ...
OMIM

Malignant atrophic papulosis (Degos disease) Sources
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Edited by Aldo Campana, March 13, 2003

12. Eastern Mediterranean Health Journal, Vol. 6 No. 1, Degos Disease In A 24-year-o
Introduction. Degos disease (malignant atrophic papulosis) is a rare disease thataffects skin and the gastrointestinal, ocular and central nervous systems.
http://www.emro.who.int/Publications/EMHJ/0601/25.htm
Degos disease in a 24-year-old Jordanian male R.M. Al-Smadi, F. Abu-Jamous and I. Omeish Volume 6, Issue 1, 2000, Page 194-196 Introduction Degos disease (malignant atrophic papulosis) is a rare disease that affects skin and the gastrointestinal, ocular and central nervous systems. The disease is characterized by papules that develop porcelain-white centres and telangiectatic borders. The etiology of the disease is unknown; however, the immediate cause may lie in impaired endothelial function or abnormal coagulation (fibrinolytic activity). Viral causes have been implicated. Histopathological findings are wedge-shaped areas of dermal necrosis covered by markedly atrophic malpighian layers and collagen with a smudged appearance. Complications of the disease include peritonitis, intestinal perforation and, less frequently, cerebral infarction. There is no effective treatment; however, antiplatelet therapy may be beneficial for patients in whom platelet aggregation is impaired. Prognosis is poor when systemic involvement is found, but seems to be good in benign forms.

13. PaperChase Search Page (custom)
Degos' disease (malignant atrophic papulosis) is a rare multisystemic disease with characteristic cutaneous lesions,
http://www.paperchase.com/docs/R1603/F205201/B8.htm
Reference 6 of 210) Flag for Printing
Calcific constrictive pericarditis in Degos' disease.
McLoud TC, Munechika H, Shaw R, Putman CE South Med J (1978 May) 71(5):609-11 ISSN: 0038-4348 Calcinosis
Pericarditis, Constrictive
Skin Diseases
Adult
Case Report
Female
Human
Pleural Effusion
Vasculitis
Abstract
A 31-year-old woman presented with symptoms and signs of constrictive pericarditis. She had a history of Degos' disease, a rare disorder characterized by skin and bowel lesions thought to be secondary to vasculitis. A chest roentgenogram showed Top of Abstract extensive calcification of the pericardium. Although pleural and pericardial involvement has been reported in this disease, constrictive pericarditis is most unusual, and radiographically demonstrable calcification of the pericardium has not been reported previously. (Reference 7 of 210) Flag for Printing
Intrathoracic manifestations of Degos' disease (malignant atrophic papulosis).
Pierce RN, Smith GJ Chest (1978 Jan) 73(1):79-84 ISSN: 0012-3692 Skin Diseases Thoracic Diseases Adult Case Report Chronic Disease Female Human Pericarditis Pleurisy Skin Thrombosis Abstract Degos' disease (malignant atrophic papulosis) is a rare multisystemic disease with characteristic cutaneous lesions, abdominal symptoms and often rapidly fatal course. 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

14. Dermatology Differential Diagnosis By Morphology
Lichen sclerosis et atrophicus; Atrophic morphea; Lichen planus (atrophicvarient); Degos disease malignant atrophic papulosis. Acral Scleroatrophy.
http://tray.dermatology.uiowa.edu/DDX/scar-atrophy.html
Dept. of Dermatology - University of Iowa College of Medicine
D ermatology D ifferential D iagnosis by M orphology
Scar / Sclerotic / Atrophy Lesions
Atrophic White Spots
Acral Scleroatrophy
Epidermal Atrophy
Dermal Atrophy ...
Subcutaneous Atrophy
Return to Dermatology's Home page.
Return to Differential Diagnosis by Morphology
Please read
Under Construction
Atrophic White Spots
  • Lichen sclerosis et atrophicus
  • Atrophic morphea
  • Lichen planus (atrophic varient)
  • Degos disease - Malignant atrophic papulosis
  • Acral Scleroatrophy
  • Acrodermatitis chronica atrophicans (Infection with Borrelia burgdorferi
  • Acrogeria
  • Scleroderma
  • Epidermal Atrophy
  • Steroid induced atrophy
  • Senile atrophy
  • Mycosis fungoides
  • Poikilodermas
    • Poikiloderma atrophicans vasculare ( +/- Reticulosis [ e.g. M.F. ] )
    • Poikiloderma of Civatte
    • Poikiloderma congenitale
    • Rothmund-Thomson syndrome
    • Dermatomyositis
    • Lupus erythematosus
    • Lichen planus erythematosus
    • Scleroderma (rare)
  • Acrogeria / Progeria / Werner's syndrome
  • Dermal Atrophy
  • Anetoderma
    • Jadassohn-Pellizari type (Inflammatory precursor)
    • Schwenniger-Buzzi type (No precursor, arises
  • 15. GASNet Anesthesiology: Köhlmeier - Degos Disease
    Köhlmeier Degos Disease. - malignant atrophic papulosis -. ·. Occlusive vasculitis
    http://gasnet.med.yale.edu/pediatric-syndromes/kohlmeier-degos.php

    16. Health Library - Degos Disease
    Synonyms. Degos Syndrome; DegosKohlmeier Disease; Kohlmeier-Degos Disease;malignant atrophic papulosis. Disorder Subdivisions. None. General Discussion.
    http://health_info.nmh.org/Library/HealthGuide/IllnessConditions/topic.asp?hwid=

    17. Springer LINK Der Hautarzt - Abstract Volume 53 Issue 1 (2002)
    Translate this page malignant atrophic papulosis not always malignant! C. Mensing, H. Mensing. A patientdeveloped malignant atrophic papulosis with only cutaneous manifestation.
    http://link.springer-ny.com/link/service/journals/00105/bibs/2053001/20530042.ht

    18. SpringerLink: Der Hautarzt - Abstract Volume 52 Issue 8 (2001) Pp 734-737
    Translate this page farbbildkasuistik Papulosis maligna atrophicans (Köhlmeier-Degos) Diagnose,Therapie, Verlauf malignant atrophic papulosis (Köhlmeier-Degos).
    http://link.springer-ny.com/link/service/journals/00105/bibs/1052008/10520734.ht
    Der Hautarzt
    ISSN: 0017-8470 (printed version)
    ISSN: 1432-1173 (electronic version) Table of Contents Abstract Volume 52 Issue 8 (2001) pp 734-737
    farbbildkasuistik
    Diagnose, Therapie, Verlauf
    C. Vicktor, U. Schultz-Ehrenburg
    Dermatologische Klinik des Klinikum Buch, Berlin
    Zusammenfassung Abstract Keywords Article in PDF format (179 KB) Online publication: July 25, 2001
    SpringerLink Helpdesk

    19. PaperChase Search Page (custom)
    90269270 (Reference 46 of 210) Flag for Printing. An atypical paediatriccase of malignant atrophic papulosis (KohlmeierDegos disease).
    http://www.paperchase.com/docs/R1603/F205201/B16.htm
    Reference 46 of 210) Flag for Printing
    An atypical paediatric case of malignant atrophic papulosis (Kohlmeier-Degos disease).
    Barabino A, Pesce F, Gatti R, Colotto P, Nobili F, Colacino R, Giampalmo A Eur J Pediatr (1990 Apr) 149(7):457-8 ISSN: 0340-6199 Arterial Occlusive Diseases
    Cerebral Ischemia
    Malabsorption Syndromes
    Case Report
    Child
    Fingers
    Human
    Male
    Raynaud's Disease
    Skin Ulcer
    Abstract A new case of malignant atrophic papulosis (Kohlmeier-Degos disease) is reported. Vascular symptoms began at 17 months of age with cerebral ischaemia and progressive involvement of fingers and toes with torpid ulcers and apical necrotic amputations. At 6 years of age he developed chronic intestinal ischaemia with malabsorption and a new cerebral attack; in spite of Top of Abstract anti-aggregant therapy the disease progressed and he died 7 months after diagnosis from a third cerebral ictus. Since the typical skin lesions of the disease were absent, the diagnosis was made on the basis of a pathological pattern of an occluded biopsied artery. The elder brother presents clinical and instrumental vascular involvement without cutaneous lesions and could be slightly affected. III Paediatric Department G Gaslini Scientific Institute Genova Italy.

    20. Dorlands Medical Dictionary
    malignant atrophic papulosis, an often fatal disease occurring most often in men,characterized by endovasculitis of the skin, gastrointestinal tract, and
    http://www.mercksource.com/pp/us/cns/cns_hl_dorlands.jspzQzpgzEzzSzppdocszSzuszS

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