1. Degos Disease Advice, support, news, and a forum for patients with degos disease (MalignantAtrophic Papulosis), their families and carers and medical advisers. http://www.degosdisease.com/

Degos Disease Home 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 Charit©-Universit¤tsmedizin Berlin There is information and space for patients, their carers and relatives, their doctors and researchers. The Degos Patients' Support Network is a contact group, a support group and an information hub for those affected by Degos disease. Medical professionals who are seeking help will find information, links to medical sites and to ongoing research. As Degos disease is an extremely rare disease, we welcome input from those affected by it. We will always listen. This site is funded by the private investment of Judith and Alan Calder and is run with no subscriptions, fees or advertising revenue of any description. We subscribe to the HONcode principles for healthcare information on the Internet. Verify here This site is listed in the University of Nottingham's OMNI catalogue of hand-selected and evaluated online medical information.

2. Degos Disease Synonyms, appearance and prognosis. 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 AGE Usually 4-6th decades Malignant atrophic papulosis in an infant. Torrelo A, Sevilla J, Mediero IG, Candelas D, Zambrano A. Department of Dermatology, Hospital del Nino Jesus, Menendez Pelayo 65, 28009 Madrid, Spain. Br J Dermatol. 2002 May;146(5):916-8. Abstract quote We report a 7-month-old girl with malignant atrophic papulosis (Degos' disease). She also showed spontaneous aggregation of platelets. A good clinical response was obtained by treatment with aspirin and dipyridamole.

3. EMedicine - Degos Disease : Article By Noah S Scheinfeld, MD, JD, FAAD Article by Noah S Scheinfeld, MD, JD, FAAD. Background, pathophysiology, causes,and treatments. http://www.emedicine.com/derm/topic931.htm

(advertisement) Home Specialties Resource Centers CME ... Patient Education Articles Images CME Patient Education Advanced Search Consumer Health Link to this site Back to: eMedicine Specialties Dermatology Diseases Of The Vessels Degos Disease Last Updated: February 3, 2005 Rate this Article Email to a Colleague Synonyms and related keywords: 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: American Academy of Dermatology Editor(s): Franklin Flowers, MD , Professor, Department of Medicine and Otolaryngology, University of Florida College of Medicine; David F Butler, MD Jeff Miller, MD , Assistant Professor, Department of Dermatology, Penn State University, Milton S Hershey Medical Center; Joel M Gelfand, MD, MSCE

4. 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

5. Degos Disease LUPUS VARIANT Degos' Disease A Distinctive Pattern of Disease, Chiefly of Lupus Erythematosus, And Not a Specific Disease per se. http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

6. What Is Degos? - DegosDisease.com degos disease Degos Patients Support Network website. http://www.degosdisease.com/information/forpatients/whatisdegos.htm

Degos Disease What is Degos? Degos Diesease seems to be a disease of the blood vessels - a thrombotic vasculopathy Cells in the linings of the walls of the medium and small veins and arteries under the skin swell when they become inflamed. This causes the blood flow to be restricted. Where this happens, spots (lesions) appear on the skin. They are small and red, slightly raised.. As they develop, the centre becomes dry and white (atrophic). Sometimes the spots itch. You can see some typical lesions in the picture inset on the left and at the following site: http://tray.dermatology.uiowa.edu/Degos01.htm In some people, the disease stays at this stage and other symptoms do not develop. We know of one patient who was diagnosed in 1973 and is still well and working full-time. Be aware that information on some sites is inaccurate . You will read statistics and numbers which might scare you - but they are often based on projections and on published case reports. There are lots of Degos patients whose cases haven’t been written up and who are alive and well. There are also lots of confident assertions which just aren’t true! For example: “This disease affects mainly young men” - not in our experience! Or “The lesions don’t appear on the soles of the feet” - wrong again! Percentages and actual numbers can’t be right, as no-one has compared living patients until now. Take it all with a pinch of salt and add

7. Degos Disease (malignant Atrophic Papulosis). DermNet NZ What is the cause of degos disease? The underlying cause of the occlusion of the blood vessels in degos disease is unknown. http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

8. Degos Disease degos disease is a rare systemic disorder that affects small and medium sized arteries, causing them to become blocked (occlusive arteriopathy) . http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

9. EMedicine - Reticulate Pigmented Anomaly : Article By Alexander Zajchenko, MD Synonyms and related keywords Dowlingdegos disease, dark dot disease, DowlingDegos Bedlow AJ, Mortimer PS Dowling-degos disease associated with http://www.emedicine.com/derm/topic776.htm

(advertisement) Home Specialties Resource Centers CME ... Patient Education Articles Images CME Patient Education Advanced Search Consumer Health Link to this site Back to: eMedicine Specialties Dermatology Pediatric Diseases Reticulate Pigmented Anomaly Last Updated: February 25, 2005 Rate this Article Email to a Colleague Synonyms and related keywords: Dowling-Degos disease, dark dot disease, Dowling Degos Ossipowski disease, DDD AUTHOR INFORMATION Section 1 of 9 Author Information Introduction Clinical Differentials ... Bibliography Author: Alexander Zajchenko, MD , Head, Professor, Department of Skin and Venereal Diseases, Medical University of Lviv, Ukraine Coauthor(s): Borys Savchyk, MD, PhD , Director, Molecular Biology Institute, Medical University of Lviv, Ukraine; Robert A Schwartz, MD, MPH , Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School Editor(s): Mark A Crowe, MD

10. Degos Disease - Advice, Support And A Forum For Degos Patients Advice, support, news, and a forum for patients with degos disease (Malignant Atrophic Papulosis), their families and carers and medical advisers. http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

11. EMedicine - Degos Disease Article By Noah S Scheinfeld, MD, JD degos disease In 1941, in an article entitled http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

12. Reticular Melanotic Hypermelanoses It seems likely that Dowlingdegos disease, and Kitamura s reticulate Is Dowling-degos disease the same disease as Kitamura s reticulate 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 Reticulate acropigmentation of Kitamura: first case reports from Nepal. Joshi A Sah SP Department of Dermatology and Venereology, Himalayan Institute of Medical Sciences, Jolly Grant, Dehradun-248140, Uttar Pradesh, India. J Dermatol. 2000 Nov;27(11):745-7. Abstract quote Two young Nepalese females, aged 34 and 17 years, presented with multiple small brown macules in a reticulate pattern on the distal extensor aspects of their limbs. The first patient's daughter also had similar lesions. The second patient had similar lesions on her eyelids and palmar pits as well. The clinical diagnosis of reticulate acropigmentation of Kitamura (RAK) was made in both the patients and was confirmed histopathologically. These are the first case reports of this condition from Nepal. DISEASE ASSOCIATIONS CHARACTERIZATION HIDRADENITIS SUPPURATIVA Hidradenitis suppurativa

13. Degos Disease - 1 Degos' Disease Malignant Atrophic Papulosis. Return to Image Index page. Return to Image Index page. Return to Dermatology's Home page. http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

14. Degos Disease (malignant Atrophic Papulosis). DermNet NZ Authoritative facts about the skin from the New Zealand Dermatological Society. http://dermnetnz.org/systemic/degos.html

@import url("/common/screen.css"); DermNet NZ Ad Authoritative facts about the skin from the New Zealand Dermatological Society Incorporated 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).

15. Hodgers.com hodgers.com website of Tom Hodgkinson http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

16. Degos Disease - 1 Dept. of Dermatology University of Iowa College of Medicine. 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

17. Malignant Atrophic Papulosis (Degos Disease) degos disease (malignant atrophic papulosis) degos disease (malignant atrophic papulosis) degos disease (malignant atrophic papulosis) http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

18. Degos Disease Degos Syndrome Degos-Kohlmeier degos disease Degos Syndrome DegosKohlmeier Disease Kohlmeier-degos disease Malignant Atrophic Papulosis degos disease Degos http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

19. DEGOS DISEASE: Contact A Family - For Families With Disabled Children: Informat Contact a Family is a UK charity for families with disabled children. We offerinformation on specific conditions and rare disorders. http://www.cafamily.org.uk/Direct/d19.html

printer friendly DEGOS DISEASE home how we can help medical information index of conditions ... how you can help Did you find this page helpful? yes no Degos disease: Malignant Atrophic Papulosis: Kohlmeier-Degos disease Degos disease is a rare inherited condition first described by Kohlmeier in 1941 and Degos in 1942. There are thought to have been about 150 cases of the condition reported in medical literature. The most severe form is Systemic Degos disease affecting children and adolescents with most cases occurring in young adults. Benign Degos disease also affects adults. Systemic Degos disease affects a number of body systems: Skin - pink or red papules (solid raised lesions) primarily on the trunk and limbs healing to leave white scars; Gastrointestinal - abdominal pain, nausea, vomiting, diarrhoea or constipation and, in the later stages, intestinal perforation and haemorrhage may occur; Neurological - manifestations involve the peripheral and central nervous systems leading to headaches, dizziness, seizures, hemiplegia (total or partial paralysis of one side of the body), aphasia (loss or impairment of the power to use or comprehend words), paraplegia (paralysis of the lower half of the body), and gaze palsy (partial or complete inability to move the eyes to all directions of gaze); Ocular - ptosis (drooping of the upper eyelid), optic neuritis (inflammation of optic nerves), diplopia (double vision) and visual field defects may occur.

20. A Family With An Overlap Of Acropigmentation Of Kitamura And A family with an overlap of acropigmentation of kitamura and dowling degos disease Indian Journal of Dermatology. 1999 OctDec; 44(4) 202-4 http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

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