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         Kohlmeier-degos Disease:     more detail

41. EMedicine - Malignant Atrophic Papulosis : Article Excerpt By: Hemant Pande, MD
and Related Terms MAP, kohlmeierdegos syndrome, Degos disease, vascular occlusive disorders, thromboangiitis
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.

42. Re: Dowling Degos
The other name for Degos disease is Kohlmeier, or kohlmeierdegos.Also, it'sknown as Malignant Atrophic Papulosis of Degos. Check and see ..
http://www.hodgers.com/degos/messages/14.shtml
Re: Dowling Degos
Follow Ups Post Followup Degos Disease Message Board FAQ Posted by Judith Calder on August 06, 1902 at 06:22:40: In Reply to: Dowling Degos posted by Tim on August 05, 1902 at 12:19:44: Hi there - thank you for posting the message.
If your wife and her relatives have Dowling-Degos disease, I'm afraid it is a completely different one from this. The other name for Degos disease is Kohlmeier, or Kohlmeier-Degos.Also, it's known as Malignant Atrophic Papulosis of Degos. Check and see.......
Follow Ups:

Post a Followup Name:
E-Mail: Subject: Comments:
: Hi there - thank you for posting the message. : If your wife and her relatives have Dowling-Degos disease, I'm afraid it is a completely different one from this. The other name for Degos disease is Kohlmeier, or Kohlmeier-Degos.Also, it's known as Malignant Atrophic Papulosis of Degos. Check and see....... Optional Link URL:
Link Title:
Optional Image URL: Follow Ups Post Followup Degos Disease Message Board FAQ

43. Re: Dowling Degos
The other name for Degos disease is Kohlmeier, or kohlmeierdegos.Also, it's knownas Malignant Atrophic Papulosis of Degos. Check and see .. Follow Ups
http://www.hodgers.com/degos/messages/33.shtml
Re: Dowling Degos
Follow Ups Post Followup Degos Disease Message Board FAQ Posted by Flavia Risso on October 28, 1902 at 19:16:19: In Reply to: Re: Dowling Degos posted by Judith Calder on August 06, 1902 at 06:22:40: : Hi there - thank you for posting the message.
: If your wife and her relatives have Dowling-Degos disease, I'm afraid it is a completely different one from this. The other name for Degos disease is Kohlmeier, or Kohlmeier-Degos.Also, it's known as Malignant Atrophic Papulosis of Degos. Check and see.......
Follow Ups:
Post a Followup Name:
E-Mail: Subject: Comments:
: : Hi there - thank you for posting the message. : : If your wife and her relatives have Dowling-Degos disease, I'm afraid it is a completely different one from this. The other name for Degos disease is Kohlmeier, or Kohlmeier-Degos.Also, it's known as Malignant Atrophic Papulosis of Degos. Check and see....... Optional Link URL:
Link Title:
Optional Image URL: Follow Ups Post Followup Degos Disease Message Board FAQ

44. ȍ˟
12, /, /, kohlmeierdegos Syndrome/Thrombangiitis Cutaneointestinalis Disseminata/Malignant Kawaskaki's disease/mucocutaneous lymph node syndroem/juvenile
http://admin.koreahospital.com/newdic3/list.asp?wordid=25

45. GASNet Anesthesiology: Köhlmeier - Degos Disease
Köhlmeier Degos disease (Malignant Atrophic Papulosis) - symptoms and caveats
http://gasnet.med.yale.edu/pediatric-syndromes/kohlmeier-degos.php

46. Case Records Of The Massachusetts General Hospital -- NEJM 1995; 332: 452-459
and geographic pattern, include Takayasu's arteritis; Behcet's disease, which involves presenceof antiphospholipid antibodies; and the kohlmeierdegos syndrome
http://med.hallym.ac.kr/~medline/public/1995/0332/0007/0452/2.htm
NEJM Home Table of Contents Previous Article Next Article
Weekly Clinicopathological Exercises: Case 5-1995: A 73-Year-Old Man with Focal Brain Lesions and Peripheral-Nerve Disease
Differential Diagnosis
    *Neurologist-in-chief, New England Medical Center; professor and chairman of neurology, Tufts University School of Medicine. I shall now analyze the abnormalities of the peripheral and central nervous system. The loss of ankle reflexes, decreased vibratory sensation in the feet, slowed motor- and sensory-nerve conduction velocities, and lack of sensory-nerve action potentials indicate a peripheral neuropathy. The involvement of both legs and arms and the sensory and reflex loss that was principally distal in the limbs suggest a diffuse peripheral polyneuropathy, although the severe proximal arm and leg weakness was atypical of that disorder. Some electrophysiologic abnormalities were asymmetric. Thigh pain and muscle tenderness were prominent. The disease may have involved the muscles directly as well, although muscle-enzyme levels were normal, or it may have affected multiple proximal-nerve portions in the arms and thighs, causing a severe mononeuritis multiplex with secondary paralysis of the proximal limb muscles. The drift of the left arm observed on the initial examination indicates asymmetric brain or nerve involvement. A CT scan showed a low-attenuation lesion in the right parieto-occipital region, and an MRI scan showed an additional small lesion in the right midbrain. Then ptosis of the right eye and a right internuclear ophthalmoplegia developed, indicating a focal lesion in the tegmentum of the right pons or the midbrain. Later, a left hemiplegia appeared and then improved, and a right extensor plantar response was found. Thus, at least four focal brain lesions developed quickly and resolved. The patient also had intermittent diffuse encephalopathy characterized by sleepiness, difficulty with concentration and attention, lethargy, and myoclonus, with diffuse slowing shown by an electroencephalographic study. The onset and course of the brain lesions and their locations are typical of transient ischemia and infarction, which were probably due to vascular disease.

47. Baylor Neurology Case Of The Month
CVT), Wegener's granulomatosis, Cogan's syndrome, kohlmeierdegos syndrome, systemiclupus erythematosus, polyarteritis nodosa, sarcoidosis, Crohn’s disease.
http://www.bcm.tmc.edu/neurol/challeng/pat45/summary.html
Patient #45
Summary and Discussion
S. Tummala, M.D.
Diagnosis: Intracranial Hypertension due to Sigmoid Sinus Thrombosis CASE SUMMARY: The 6 year old boy described in this case, whose clinical symptoms included only headache without papilledema or focal neurological signs, had isolated intracranial hypertension documented by increased opening pressure at lumbar puncture. Substantial clinical improvement in symptoms was observed following the lumbar puncture, as is frequently observed in cases of intracranial hypertension. However, the age and gender of the patient are somewhat atypical in many series of patients with idiopathic intracranial hypertension (pseudotumor cerebri), and the rapid onset of symptoms (over a few days) also raised a concern that the elevated CSF pressure might be secondary to an identifiable cause. MRI and MRV imaging showed evidence of thrombosis of the sigmoid sinus on one side, with normal drainage of the remaining venous system, and no evidence of venous infarction. No evidence of systemic vascular occlusions was evident on the history and general examination. DISCUSSION: The earliest description of cerebral vein and dural sinus thrombosis is attributed to Ribes in 1825. The true incidence of this disorder is unknown, as many cases are asymptomatic or difficult to distinguish from benign symptoms. However, evidence of cerebral vein or sinus thrombosis was documented in 9 percent of 182 consecutive autopsies, with a 2:1 female preponderance. Furthermore, sinovenous thrombosis was diagnosed in 3.75 percent of a pediatric population undergoing angiography. These data suggest that the

48. Index List [K]
.. to Knee Replacement. KO. From kohlmeierdegos Syndrome .. to Kostmann Syndrome.KR. From Krabbe disease .. to Krukenberg Tumor. KS. From KSHV ..
http://www.outlinemed.com/demo/idxmeta/idxlistK.htm
K
  • K
  • From K ... ... to K
  • K-
  • From K-F Ring ... ... to K-ras
  • KA
  • From Kabikinase ... ... to Kayser-Fleischer Rings
  • KE
  • From Keflex ... ... to Ketorolac tromethamine
  • KF
  • From KF Rings ... ... to KF Rings
  • KI
  • From Kidney ... ... to Kininase
  • KL
  • From Klatskin Tumor ... ... to Klonopin
  • KN
  • From knee arthroscopy ... ... to Knee Replacement
  • KO
  • From Kohlmeier-Degos Syndrome ... ... to Kostmann Syndrome
  • KR
  • From Krabbe Disease ... ... to Krukenberg Tumor
  • KS
  • From KSHV ... ... to KSHV
  • KU
  • From Kupfer cell ... ... to kuru
  • KW
  • From Kwell ... ... to Kwell
  • KY
  • From Kytril ... ... to Kytril

    49. Euskaraz.net - Top
    Similar pages GIHEMORRHAGE 22. kohlmeierdegos syndrome. 23. 46 197, 1964 (Kaposi's sarcoma). * Samitz - Skin2 243, 1963 (skin manifestations in GI disease). ** Bean - Trans. Am. Clin.
    http://www.euskaraz.net/Loturak/Top/Health/Conditions_and_Diseases/Rare_Disorder
    IDIOMA Euskara Castellano Mapa Loturak Albisteak Donostia Gaur ... Gorago
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    Bilatzaileak
    Donostia Erakundeak ... Zerbitzuak Aurki bilatzaileko loturak
    Loturak
    Top Health Conditions and Diseases ... Rare Disorders Degos (4)
    Atal honetan Lotura bat gehitu Lotura bat eguneratu Azalpena Editore bihur zaitez ... editatu
    Editoreak Atal honek editorea behar du
    Ikus halaber: Guneak:
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    udala_euskara@donostia.org

    50. BRAIN PROBLEM
    with CVT), Wegener's granulomatosis, Cogan's syndrome, kohlmeierdegos syndrome,systemic lupus erythematosus, polyarteritis nodosa, sarcoidosis, Crohn disease.
    http://www.vghtc.gov.tw:8082/neuro/case-report-CVT.HTM
    Admisson Note
    Intern §fÂE«Â
    General Data
    Name: ªLO¥É Age: 36 y/o Sex: F Occupation: housewife Marital status: married Bed No:W41-153 Chart No:455230B Date of admission: 91/04/12 Date of history taking:91/04/12 Chief Complaint Headache for 9 days , tinnitus over right ear for 1 day. Present Illness According to the statements of the patient and herself, she had headache over right head at night after she received on 91.03.25. She didn¡¦t drink much water at that day because she was on diet. Headache improved after she drank some water. Headache was also noted on 3/26. On 3/27, severe exploseive, thrombling headache was noted over the whole head was noted. She was sent to ªL·s Hosp. for help and was admitted. Brain CT was done over there. After medical treatment, symptoms improved. She was discharged after about 5 days later. She visited our OPD for help on 4/8, when her headache recovered. Straight sinus and transverse sinus thrombus were suspected by brain CT over ªL·s Hosp. MRI was done later. Tinnitus over right ear was noted in recent 2 days. She visited our OPD on 04/12 and was admitted to our Meta ward for further evaluation and treatment. Past History 1. Hypertension: denied

    51. Text27
    of the cytotoxic agents b. Very effective in preventing relapse of disease c. Usu ANCAtiters predicted relapse in many patients F. kohlmeierdegos Syndrome 1
    http://www.ankara.edu.tr/medicine/internal_medical/internal_diseases/avicenna/ta
    POLYARTERITIS NODOSA (PAN) and RELATED
    SYNDROMES See Card "ANCA Related Diseases"
    A. Characteristics
    1. Inflammation affects medium and small arteries; usually in middle aged men.
    2. Produces aneurysms at branching points; 70% patients have intra-renal aneurysms
    3. Patients usually very sick with significant systemic symptoms, malaise and weight loss
    4. Types
    a. Classical PAN - medium and small arteries only
    c. By these definitions, Polyangiitis is much more common than true PAN
    d. Churg-Strauss Syndrome is a PAN varient with asthma, atopy, and eosinophilia
    B. Pathophysiology 1. Anti-neutrophil cytoplasmic antibody (ANCA) positive ~50% [1] a. Most patients have perinuclear ANCA pattern b. C-ANCA often positive if lungs are involved and/or with Wegener's Granulomatosis c. C-ANCA is much more common in Wegener's Granulomatosus (see below) d. P-ANCA is usually directed against Myeloperoxidase (MPO) in PAN e. P-ANCA may be found in Churg-Strauss disease and ~50% of IBD patients f. P-ANCA also found in rapidly progressive glomerulonephritis and other disease

    52. Syndrome From OMD
    KocherDebre-Semelaigne syndrome; Koenig's syndrome; kohlmeier-degos syndrome; Korsakoff's prolapsesyndrome; mixed connective tissue disease; Mobius' syndrome
    http://med.fnetsys.net/syndrome.htm
    On-Line Medical Dictionary
    syndrome
    Related entries include: 17-hydroxylase deficiency syndrome Aarskog syndrome abdominal muscle deficiency syndrome abstinence syndrome ... Zollinger-Ellison syndrome Published at the Dept. of Medical Oncology, University of Newcastle upon Tyne

    53. EMedicine - Malignant Atrophic Papulosis : Article By Hemant Pande, MD
    An atypical paediatric case of malignant atrophic papulosis (Kohlmeier Degos disease). Eur J Pediatr 1990 Apr; 149(7)
    http://www.emedicine.com/med/topic2943.htm
    (advertisement) Home Specialties CME PDA ... Patient Education Articles Images CME Patient Education Advanced Search Link to this site Back to: eMedicine Specialties Medicine, Ob/Gyn, Psychiatry, and Surgery Gastroenterology
    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

    54. Kohlmeier – Degos Disease
    Expand your medical search to other sites by only one click …. For MedicalProfessionals only. Kohlmeier – degos disease,, Print this article,
    http://www.amershamhealth.com/medcyclopaedia/Volume IV 1/KOHLMEIER DEGOS DISEAS
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    *For Medical Professionals only, registration required Kohlmeier – degos disease, rare vasculitis characterized by skin lesions as well as malabsorption, bowel ischaemia and perforation.
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    55. Browse By Letter 'K'
    syndrome Kocher – lorenz fracture Koerner's septum Kohler's disease (Volume III1) Kohler's disease (Volume VII) Kohlmeier – degos disease Kohn, pores of
    http://www.amershamhealth.com/medcyclopaedia/indexk.asp
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    56. PaperChase Search Page (custom)
    Hidradenitis suppurativa, Dowling Degos disease and perianal squamous cell with Dowling Degos disease and perianal squamous cell with Dowling Degos disease are reviewed and the
    http://www.paperchase.com/docs/R1603/F205201/B12.htm
    Reference 26 of 210) Flag for Printing
    Hidradenitis suppurativa, Dowling Degos disease and perianal squamous cell carcinoma.
    Li M, Hunt MJ, Commens CA Australas J Dermatol (1997 Nov) 38(4):209-11 ISSN: 0004-8380 Acanthosis Nigricans
    Anal Gland Neoplasms
    Carcinoma, Squamous Cell
    Hidradenitis Suppurativa
    Hyperpigmentation
    Aged
    Animal
    Axilla
    Biopsy, Needle
    Case Report
    Diagnosis, Differential Follow-Up Studies Groin Human Male Perineum Skin Transplantation Medline Database Healthstar Database Cancerlit Database Abstract 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 Top of Abstract 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. Department of Dermatology Westmead Hospital Sydney New South Wales Australia.

    57. Untitled
    disease.html. C\ Inetpub\ web. amershamhealth. com\ medcyclopaedia\ Volume IV 1\KOHLMEIER DEGOS disease.asp does
    http://www.amersham-health.com/medcyclopaedia/Volume%20IV%201/KOHLMEIER%20DEGOS%
    Input to mappath=medcyclopaedia/Volume%20IV%201/KOHLMEIER%20DEGOS%20DISEASE.html

    58. ILDS: ICD-10 - By Code [I00 - I972]
    I77800 Malignant atrophic papulosis, I77800 Syndrome Kohlmeier Degos, I77800Papulosis malignant atrophic, I77800 disease Degos, I77800 Degos disease,
    http://www.ilds.org/icd10/bycode/I.html
    Application to Dermatology of ICD-10 Alphabetized by ICD Code starting with I:
    Return to Alphabetical listing...
    ICD Code: Disorder Rheumatic fever Fever rheumatic Arteriosclerosis of arteries of extremities Atherosclerosis of arteries of extremities Atherosclerotic gangrene Gangrene atherosclerotic Disease Raynaud's Raynaud's disease Phenomenon Raynaud's Raynaud's phenomenon Raynaud's gangrene Gangrene Raynaud's Buerger's disease Disease Buerger's Thromboangiitis obliterans Acrocyanosis Nodular erythrocyanosis Erythrocyanosis with nodules Erythrocyanosis nodular Erythrocyanosis Erythermalgia Erythralgia Erythromelalgia Mitchell's syndrome Syndrome Mitchell's Livedo racemosa Livedo reticularis Livedo annularis Arteriovenous aneurysm acquired Aneurysm arteriovenous acquired Pseudo Kaposi sarcoma Stewart Blufarb syndrome Syndrome Stewart Blufarb Syndrome Degos Malignant atrophic papulosis Syndrome Kohlmeier Degos Papulosis malignant atrophic Disease Degos Degos disease Degos syndrome Kohlmeier Degos syndrome Telangiectasia hereditary hemorrhagic Hereditary hemorrhagic telangiectasia Osler Weber Rendu syndrome Rendu Osler syndrome Syndrome Osler Weber Rendu Syndrome Rendu Osler Angiomatosis Osler Weber Rendu Spider telangiectasia Spider nevus Spider arterial Spider angioma Nevus stellar Nevus spider Nevus araneus Angioma spider Telangiectasia spider Arterial spider Senile hemangioma Senile angioma Hemangioma senile Angioma senile Arborising telangiectasia Telangiectasia arborising Generalised essential telangiectasia

    59. ILDS: ICD-10 - By Disorder [Kaffir Pox - Kyrle's Disease]
    L67801 Knotting of hair, M721 Knuckle pad, Q81000 Koebner's disease,I77800 Kohlmeier Degos syndrome, Q84841 Kohlschutter syndrome,
    http://www.ilds.org/icd10/bydisorder/K.html
    Application to Dermatology of ICD-10 Alphabetized by Disorder starting with K:
    Return to Alphabetical listing...
    ICD Code: Disorder Kaffir pox Kahler's disease Kairo cancer Kala azar Kallin's syndrome Kang cancer Kangri cancer Kaposi's disease (xeroderma pigmentosum) Kaposi's sarcoma (NOS) Kaposi's sarcoma in HIV disease Kaposi's sarcoma of skin Kaposi's varicelliform eruption Kasabach Merritt syndrome Katayama disease Kawasaki disease Kelis Kelly Paterson syndrome Keloid Keloid Addison's Keloidal acne Keloidal blastomycosis Kenya tick typhus Keratitis rosaceous Keratitis sicca Keratoacanthoma Keratoacanthoma generalised eruptive Keratoconjunctivitis sicca Keratoderma acquired Keratoderma blenorrhagicum Keratoderma circumscribed palmoplantar Keratoderma climactericum Keratoderma congenital (NOS) Keratoderma diffuse palmoplantar Keratoderma focal acral Keratoderma hereditaria mutilans Keratoderma lymphedematous Keratoderma mutilating Keratoderma palmoplantar Howel Evans Keratoderma palmoplantar Meleda Keratoderma palmoplantar Olmsted Keratoderma palmoplantar Papillon Lefevre Keratoderma palmoplantar Richner Hanhart Keratoderma palmoplantar Unna Thost Keratoderma palmoplantar Vohwinkel Keratoderma palmoplantaris transgrediens Keratoderma progressive palmoplantar Keratoderma punctata Keratoderma striate Keratoderma with carcinoma of esophagus Keratoderma with deafness Keratoderma with scleroatrophy Keratolysis exfoliativa Keratolysis pitted Keratolysis plantare sulcatum Keratolytic winter erythema

    60. Searchalot Directory For Degos
    Degos disease Judth Calder's support network for this rare vasculitis. NORD -Degos disease - Offers synonyms, a general discussion and further resources.
    http://www.searchalot.com/Top/Health/ConditionsandDiseases/RareDisorders/Degos/
    Home Search News Email Greetings Weather ... Global All the Internet About AltaVista AOL Search Ask Jeeves BBC Search BBC News Business Dictionary Discovery Health Dogpile CheckDomain CNN Corbis eBay Education World Employment Encyclopedia Encarta Excite Fast Search FindLaw FirstGov Google Google Groups Infomine iWon Librarians Index Looksmart Lycos Metacrawler Microsoft Northern Light Open Directory SearchEdu SearchGov Shareware Teoma Thesaurus Thunderstone WayBackMachine Webshots WiseNut Yahoo! Yahoo! Auctions Yahoo! News Yahooligans Zeal Sponsored Links Top Health Conditions and Diseases Rare Disorders : Degos Related Web Sites
    • Degos Disease - Judth Calder's support network for this rare vasculitis.
    • NORD - Degos Disease - Offers synonyms, a general discussion and further resources.
    • Doctors Doctor - An in depth look at degos disease including prognosis and treatment.
    • Yahoo! Groups : Degos Disease - An email group offering description of what to expect upon joining, joining instructions, post and read mail.
    Related Categories All the Internet About AltaVista AOL Search Ask Jeeves BBC Search BBC News Business Dictionary Discovery Health Dogpile CheckDomain CNN Corbis eBay Education World Employment Encyclopedia Encarta Excite Fast Search FindLaw FirstGov Google Google Groups Infomine iWon Librarians Index Looksmart Lycos Metacrawler Microsoft Northern Light Open Directory SearchEdu SearchGov Shareware Teoma Thesaurus Thunderstone WayBackMachine Webshots WiseNut Yahoo!

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