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         Degos Disease:     more detail
  1. Case of the month. (Practice Trends).(diagnosing Degos' disease): An article from: Skin & Allergy News by Nancy Walsh, 2003-06-01
  2. Derm diagnosis.(Degos disease): An article from: Family Practice News by Nancy Walsh, 2003-06-01
  3. Textbook of Malignant Haematology by Laurent Degos, David C Linch, et all 1999-01-01
  4. Dermatologie (French Edition) by Robert Degos, 1976

1. Degos Disease
Panniculitis mimicking lupus erythematosus profundus a new histopathologicfinding in malignant atrophic papulosis (degos 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. OUTLINE Epidemiology Disease Associations Pathogenesis Laboratory/Radiologic/Other Diagnostic Testing ... Internet Links
EPIDEMIOLOGY CHARACTERISTICS 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 LUPUS VARIANT
Degos' Disease: A Distinctive Pattern of Disease, Chiefly of Lupus Erythematosus, And Not a Specific Disease per se.

2. EMedicine - Degos Disease : Article By Noah S Scheinfeld, MD, JD, FAAD
degos disease. Last Updated October 13, 2003, insert. FULL DISCLAIMER.degos disease excerpt. © Copyright 2004, eMedicine.com, Inc.
http://www.emedicine.com/derm/topic931.htm
(advertisement) Home Specialties CME PDA ... Patient Education Articles Images CME Patient Education Advanced Search Link to this site Back to: eMedicine Specialties Dermatology Diseases Of The Vessels
Degos Disease
Last Updated: October 13, 2003 Rate this Article Email to a Colleague Synonyms and related keywords: DD, malignant atrophic papulosis, papulosis atrophicans maligna, Kohlmeier-Degos-Delort-Tricot syndrome, Kohlmeier-Degos syndrome, Köhlmeier-Degos' disease, Online Mendelian Inheritance in Man 602248, OMIM 602248, papuleuse maligne atrophiante, lethal cutaneous and gastrointestinal arteriolar thrombosis, fatal cutaneointestinal syndrome, thromboangiitis cutaneointestinalis disseminata, dermatite papulosquameuse atrophiante, MAP AUTHOR INFORMATION Section 1 of 10 Author Information Introduction Clinical Differentials ... Bibliography
Author: Noah S Scheinfeld, MD, JD, FAAD , Assistant Professor, Department of Dermatology, Columbia University; Head of Consultation Service, Department of Dermatology, St Luke's Roosevelt Hospital Center Noah S Scheinfeld, MD, JD, FAAD, is a member of the following medical societies:

3. NORD - National Organization For Rare Disorders, Inc.
Sample report, plus links to organisations. Fee required for full report.
http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Degos Disease

4. Degos Disease - 1
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, 1995Copyright © 1995 - 2002 Dept.
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

5. Degos Disease (malignant Atrophic Papulosis). DermNet NZ
degos disease patient information and doctors. NZ DermNet is an online dermatology resource for patients, GPs and dermatologists degos disease. degos disease. degos disease, also known as malignant atrophic papulosis is a rare disorder degos disease affects the lining of the small blood vessels resulting in
http://www.dermnetnz.org/dna.degos/degos.html
@import url("/common/screen.css");
DermNet NZ
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Authoritative facts
about the skin from the New Zealand Dermatological Society Home For patients For doctors ... Site map Search:
Home
Systemic
Degos disease
Degos disease, also known as malignant atrophic papulosis is a rare disorder. It generally occurs in young Caucasian adults. Degos disease affects the lining of the small blood vessels resulting in occlusion (blockage). The blood vessels affected include those supplying the skin, gastrointestinal tract and central nervous system.
Degos scar
Multiple lesions
New lesions
What is the cause of Degos disease?
The underlying cause of the occlusion of the blood vessels in Degos disease is unknown. Three possible mechanisms are:
  • Viral infection Disturbance in immunity Abnormality in the clotting system of blood
What are its features?
Skin lesions are the characteristic feature of Degos disease. The lesions are usually multiple and occur predominantly on the trunk and arms. They usually start as small red raised spots of 2-5mm in diameter. After a few days they enlarge and develop a central white spot that is depressed in comparison to the red skin around it. They heal leaving depressed porcelain-white scars. Gastrointestinal complications result from lack of blood supply to the lining of the gut and may lead to serious complications like perforation of the bowel. The usual symptoms are sudden onset of abdominal pain or gastrointestinal bleeding (vomiting blood or passing blood with the bowel motion).

6. EMedicine - Degos Disease : Article Excerpt By: Noah S Scheinfeld, MD, JD, FAAD
eMedicine World Medical Library. (advertisement). Excerpt from DegosDisease. Please click here to view the full topic text degos disease.
http://www.emedicine.com/derm/byname/degos-disease.htm
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Excerpt from Degos Disease
Synonyms, Key Words, and Related Terms: DD, malignant atrophic papulosis, papulosis atrophicans maligna, Kohlmeier-Degos-Delort-Tricot syndrome, Kohlmeier-Degos syndrome, Köhlmeier-Degos' disease, Online Mendelian Inheritance in Man 602248, OMIM 602248, papuleuse maligne atrophiante, lethal cutaneous and gastrointestinal arteriolar thrombosis, fatal cutaneointestinal syndrome, thromboangiitis cutaneointestinalis disseminata, dermatite papulosquameuse atrophiante, MAP
Please click here to view the full topic text: Degos Disease
Background: In 1941, in an article entitled "Multiple Hautrekrosen bei Thromboangiitis obliterans," Kohlmeier described a case of a disease that has now been termed malignant atrophic papulosis (MAP) or Degos disease (DD). Degos recognized it as a distinct clinical entity in 1942. Broadly speaking, DD is a vasculopathy or an endovasculitis. It is an occlusive arteriopathy involving small-caliber vessels. Specifically, it is a progressive, small- and medium-size arterial occluding disease, leading to tissue infarction and initially involving the skin. DD occurs both in a limited benign, cutaneous form and in a lethal multiorgan, systemic variant. In the skin, DD initially manifests with erythematous, pink or red papules. These papules heal to leave scars with pathognomonic, central, porcelain white atrophic centers. These papules usually have a peripheral telangiectatic rim.

7. Degos Disease - Advice, Support And A Forum For Degos Patients, Their Families A
Advice, support, news, and a forum for patients with degos disease (Malignant Atrophic Papulosis), their families and carers and medical advisers.
http://www.degosdisease.com
Welcome to the Degos Patients Support Network website! This website has been devised by Judith Calder, a Degos patient in Sussex, England, with the full co-operation and support of Professor Christos Zouboulis, Vice Chair of the Department of Dermatology at Charite-Universitaetsmedizin Berlin. Working with data collected from patients around the world, Professor Zouboulis will be presenting the first ever World Conference on Degos disease in Berlin in 2004. All patients and their medical advisers are encouraged to attend this event, which will be the first of its kind ever to be arranged. No-one in the history of this disease has compared living patients before. If it's your first visit to the site, take a look at the About this Site pages and whatever your interest in this condition, there should be something for you to learn or to contribute. There is information and space for patients, their carers and relatives, their doctors and researchers. There are links to other sites giving information about Degos disease. Please use the Forum and add to the FAQs (Frequently Asked Questions) . If you have answers to questions, do feel free to add them in too.

8. Reticular Melanotic Hypermelanoses
HIDRADENITIS SUPPURATIVA, Hidradenitis suppurativa, Dowling degos diseaseand perianal squamous cell carcinoma. Li M, Hunt MJ, Commens CA.
http://www.thedoctorsdoctor.com/diseases/reticular_melanotic_hypermelanoses.htm
Background These rare pigmented diseases are all characterized by a peculiar reticulate pigmentation. They are divided into various disease based upon the distribution and associated conditions. OUTLINE Epidemiology Disease Associations Pathogenesis Gross Appearance and Clinical Variants ... Internet Links
EPIDEMIOLOGY CHARACTERIZATION SYNONYMS Reticulate pigmentary disorders GEOGRAPHY More common in Asians DISEASE ASSOCIATIONS CHARACTERIZATION HIDRADENITIS SUPPURATIVA
Hidradenitis suppurativa, Dowling Degos disease and perianal squamous cell carcinoma. Li M, Hunt MJ, Commens CA. Department of Dermatology, Westmead Hospital, Sydney, New South Wales, Australia. Australas J Dermatol 1997 Nov;38(4):209-11 Abstract quote A patient with hidradenitis suppurativa (HS) is described in association with Dowling Degos disease and perianal squamous cell carcinomas. As the initial treatment for HS failed to give any satisfactory relief he sought no further medical help for the subsequent 40 years. This patient illustrates the management difficulties in HS and the need for regular surveillance to exclude the development of anogenital squamous cell carcinoma. Previous reports of hidradenitis suppurativa in association with Dowling Degos disease are reviewed and the aetiology is discussed.

9. Degos' Disease Information Diseases Database
Degos' disease Erythrokeratoderma en cocardes Kohlmeierdegos disease Malignant atrophic papulosis, Disease Database Information 4 synonyms or equivalents were found. Degos' disease. aka/or
http://www.diseasesdatabase.com/sieve/item1.asp?glngUserChoice=29425

10. Hodgers.com - Degos Disease
This website has closed. Links to relevant degos disease resources are listed below.» DegosDisease.com. » National Organisation for Rare Disorders (NORD).
http://www.hodgers.com/degos/
This website has closed.
Links to relevant Degos Disease resources are listed below. DegosDisease.com National Organisation for Rare Disorders (NORD) University of Iowa Dept. of Dermatology (pic) The Doctor's Doctor ... hodgers.com

11. DEGOS DISEASE
She was a very brave and loving woman. She was diagnosed with Degosdisease back in 2002. She withstood a long and hard battle.
http://www.degosdisease.com/guestbook/guestbook.html
Tributes appear below. You can add your own tribute This is a tribute to one of the most beautiful and loving people I have ever had the priviledge of knowing and loving. This is in memory of Shirley King. Shirley has gone on to heaven and is waiting there for the rest of us. She was a very brave and loving woman. She was diagnosed with Degos disease back in 2002. She withstood a long and hard battle. She will be missed dearly by all of her family and friends. We know there is a new "angel" in heaven, as she was an "angel" here on earth. We love you Shirley, you will always be in our hearts! Thank you for the love you gave us all through out our lives!
Missouri, USA - 17:05 on 28/01/04 Marge Potter
Some of us first met Marge back in 1971. She was a freshman at Marist. A beauty, with waist long straight hair, she was a head turner for the guys on campus. Being the ambitious person she was, Marge was working her way through college. Rather than pile up school loans, she decided to leave Marist the end of her first year and return home to Brooklyn, work for a year or two, save and later complete school.
In 1974, after attending one of Barbara Dudley's famous parties on South Hamilton St., she fell in love with the people, the apartment building and after learning of a vacancy upstairs, left a deposit for the apartment and decided to return to Poughkeepsie to complete her education.

12. Degos Disease
I am a 55 year old teacher who was diagnosed with this disease in January 1998. in Chichester, UK who correctly diagnosed degos disease. He explained the nature of
http://www.hodgers.com/degos/about_judith.shtml
PATIENT SUPPORT NETWORK AND INFORMATION RESOURCE About Judith...
I am a 55 year old teacher who was diagnosed with this disease in January 1998.
I was referred to Dr Peter Coburn, a Consultant Dermatologist in Chichester, UK who correctly diagnosed Degos Disease. He explained the nature of this extremely rare condition.
It seems that the cells in the walls of the small and medium-sized arteries and veins multiply and close off blood-supply to the skin in the first stage. In some patients the disease does not progress beyond this stage. In others lesions internally may affect the small intestine and other major organs.
I am now under the care of Dr Robin Russell-Jones at the Cromwell Hospital, London. He has prescribed 75mg Aspirin per day and 3mg per kilo bodyweight per day of Cyclosporin. This appears to be effective treatment although it is, of course, experimental.
I appear to be the only patient in Europe with this diagnosis! Surely this cannot be true!
If you - as a patient or physician - have any information or experience of this disease please contact me (see right) in order to be included on a database for the Degos Patients Network. Resources
Contact

JUDITH CALDER
Phone:
Fax: Email: judithcalder@wrencottage.co.uk

13. Degos Disease.
degos disease, also known as malignant atrophic papulosis is a rare disorder.It affects the lining degos disease. degos disease, also known
http://www.betterhealth.vic.gov.au/bhcv2/bhcarticles.nsf/pages/Degos_disease?ope

14. Skin Hair And Nails - Other Skin Conditions
the crusts, can help. degos disease degos disease, also known asmalignant atrophic papulosis is a rare disorder. It affects the
http://www.betterhealth.vic.gov.au/bhcv2/bhcarticles.nsf/pages/hc_skinhairnails_

15. Degos Disease
degos disease is a rare systemic disorder that affects small and medium sized arteries,causing them to become blocked (occlusive arteriopathy). degos disease.
http://www.bchealthguide.org/kbase/nord/nord991.htm
document.write(''); var hwPrint=1; var hwDocHWID="nord991"; var hwDocTitle="Degos Disease"; var hwRank="1"; var hwSectionHWID="nord991"; var hwSectionTitle=""; var hwSource="cn6.0"; var hwProdCfgSerNo="wsh_html_031_s"; var hwDocType="NORD";
National Organization for Rare Disorders, Inc.
Degos Disease
Important
It is possible that the main title of the report is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Synonyms
  • Degos Syndrome Degos-Kohlmeier Disease Kohlmeier-Degos Disease Malignant Atrophic Papulosis
Disorder Subdivisions
  • None
Related Disorders List
Information on the following diseases can be found in the Related Disorders section of this report:
  • Buerger's Disease Lichen Sclerosus et Atrophicus (LSA) Lymphomatoid Granulomatosis Scleroderma Vasculitis Vasculitis, Cutaneous Necrotizing
General Discussion
Degos disease is a rare systemic disorder that affects small and medium sized arteries, causing them to become blocked (occlusive arteriopathy). Degos disease usually progresses through two stages. During the first stage, characteristic skin lesions appear that may last for a period of time ranging from weeks to years. The second stage of Degos disease is most frequently characterized by lesions in the small intestine, but other organs may also be affected. Major symptoms may include abdominal pain, diarrhea, and/or weight loss. Intestinal lesions may break through the wall of the bowel (perforation), a potentially life-threatening complication. The exact cause of Degos disease is unknown.

16. Degos Disease
degos disease Important It is possible that the main title of thereport degos disease is not the name you expected. Please check
http://my.webmd.com/hw/heart_disease/nord991.asp
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  • Degos Syndrome Degos-Kohlmeier Disease Kohlmeier-Degos Disease Malignant Atrophic Papulosis
Disorder Subdivisions
  • None
General Discussion Degos disease is a rare systemic disorder that affects small and medium sized arteries, causing them to become blocked (occlusive arteriopathy). Degos disease usually progresses through two stages. During the first stage, characteristic skin lesions appear that may last for a period of time ranging from weeks to years. The second stage of Degos disease is most frequently characterized by lesions in the small intestine, but other organs may also be affected. Major symptoms may include abdominal pain, diarrhea, and/or weight loss. Intestinal lesions may break through the wall of the bowel (perforation), a potentially life-threatening complication. The exact cause of Degos disease is unknown.

17. Degos - Information / Diagnosis / Treatment / Prevention
Doctor s Doctor degos disease Synonyms, appearance and prognosis.? NORD degos disease Sample report, plus links to organisations.
http://www.healthcyclopedia.com/rare-disorders/degos.html

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Related Topics: Cardiovascular Disorders/Vascular Disorders Medical Definition: University of Newcastle-upon-Tyne Medical Dictionary: "Degos' disease" Health News: Search millions of published articles for news on Degos Modern Medicine Aging The Ardell Wellness Report HealthFacts Medical Post Medical Update Men's Health and the National Women's Health Report Note: Subscription required to access the full text of articles. Web Directory: Degos Disease Email group to share information and support. Degos Patients' Support Network Advice, support, news, and a forum for patients with Degos disease (Malignant Atrophic Papulosis), their families and carers and medical advisers. Doctor's Doctor: Degos Disease Synonyms, appearance and prognosis.

18. Degos' Disease Information Diseases Database
Degos disease,Erythrokeratoderma en cocardes,Kohlmeierdegos disease,Malignantatrophic papulosis, Disease Database Information.
http://www.diseasesdatabase.com/ddb29425.htm
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Degos' disease Information
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4 synonyms or equivalents were found. Degos' disease
aka/or
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19. Disease, Medication, Symptom Etc Database Index : K Diseases Database
phenomenon KoerberSalus-Elshnig syndrome Kohler s first disease Kohler s seconddisease see Freiberg s disease Kohlmeier-degos disease see Degos disease
http://www.diseasesdatabase.com/disease_index_k.asp
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Kabuki make-up syndrome

Kahler's disease see Myeloma
Kala-Azar see Visceral leishmaniasis
Kallmann syndrome

Kanamycin

Kandinsky syndrome

Kanner's syndrome see Autism
Kanzaki disease see Schindler disease
Kaolin

Kaposi varicelliform eruption
Kaposiform hemangioendothelioma ... Kaposi's sarcoma Karaya gum see Sterculia Karenitecin Kartagener's syndrome Karyotype disorders see Chromosomal abnormalities Kasabach-Merritt syndrome Kaschin-Beck disease Kashin-Beck disease see Kaschin-Beck disease Katayama fever see Schistosoma japonicum Kaufman-McKusick syndrome Kava root extract Kawasaki disease Kayser-Fleischer ring see Fleischer-Kayser ring Kearns-Sayre syndrome Kedani fever see Scrub typhus Keller syndrome see Opitz-Kaveggia syndrome Keloid Keloidal blastomycosis see Lobomycosis Kennedy's disease Kenny-Caffey-Linarelli syndrome Keratinocyte growth factor 2 ... Keratoacanthoma Keratoconjunctivitis sicca see Sicca syndrome Keratoconus Keratoderma blenorrhagica Keratoderma climactericum Keratoderma hereditaria mutilans see Vohwinkel's mutilating keratoderma Keratoderma with scleroatrophy see Huriez syndrome Keratoglobus see Megalocornea Keratolytic agents Keratomalacia Keratomycosis nigricans see Tinea nigra Keratopathy see Corneal pathology Keratosis extremitatum hereditaria trangrediens et progrediens see Meleda disease Keratosis follicularis (congenital) Keratosis lichenoides chronica see Lichen ruber moniliformis Keratosis obturans Keratosis palmaris et plantaris see Palmoplantar keratosis Keratosis palmoplantaris transgrediens et progrediens

20. Degos Disease,Degos Syndrome,Degos-Kohlmeier Disease,Kohlmeier-Degos Disease,Mal
degos disease,Degos Syndrome,DegosKohlmeier Disease,Kohlmeier-degos disease,MalignantAtrophic Papulosis,degos disease,Degos Syndrome,Degos-Kohlmeier Disease
http://www.icomm.ca/geneinfo/degos.htm
Degos Disease,Degos Syndrome,Degos-Kohlmeier Disease,Kohlmeier-Degos Disease,Malignant Atrophic Papulosis,Degos Disease,Degos Syndrome,Degos-Kohlmeier Disease,Kohlmeier-Degos Disease,Malignant Atrophic Papulosis,Degos Disease,Degos Syndrome,Degos-Kohlmeier Disease,Kohlmeier-Degos Disease,Malignant Atrophic Papulosis
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DISORDERS GLOSSARY Degos Disease
(as defined by the
National Organization for Rare Disorders

also known as:
Degos Syndrome
Degos-Kohlmeier Disease
Kohlmeier-Degos Disease Malignant Atrophic Papulosis
Degos Disease is a rare systemic disorder that affects small and medium sized arteries, causing them to become blocked (occlusive arteriopathy). Degos Disease usually progresses through two stages. During the first stage, characteristic skin lesions appear that may last for a period of time ranging from weeks to years. The second stage of Degos Disease is most frequently characterized by lesions in the small intestine, but other organs may also be affected. Major symptoms may include abdominal pain, diarrhea, and/or weight loss. Intestinal lesions may break through the wall of the bowel (perforation), a potentially life-threatening complication.

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