Articles & Papers

The following papers and articles are known about Degos Disease. Please let us know of any other papers by contacting us.

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2013

Malignant atrophic papulosis (Köhlmeier-Degos Disease - A Review
Link: http://www.ojrd.com/content/8/1/10/abstract
Authors: Athanasios Theodoridis1,† Email: drtheodoridis@googlemail.com Evgenia Makrantonaki1,2,† * Corresponding author Email: emakrantonaki@yahoo.com Christos C Zouboulis1*,† * Corresponding author Email: christos.zouboulis@klinikum-dessau.de 1 Departments of Dermatology, Venerology, Allergology and Immunology, Dessau Medical Center, Auenweg 38, Dessau 06847, Germany 2 Institute of Clinical Pharmacology and Toxicology, Charité Universitätsmedizin Berlin, Charitéplatz 1, 10117 Berlin, Germany
1 Departments of Dermatology, Venerology, Allergology and Immunology, Dessau Medical Center, Auenweg 38, Dessau 06847, Germany 2 Institute of Clinical Pharmacology and Toxicology, Charité Universitätsmedizin Berlin, Charitéplatz 1, 10117 Berlin, Germany
Reference:
Correspondence: <Drtheodoridis@googlemail.com, emakrantonaki@yahoo.com, christos.zouboulis@klinikum-dessau.de>

Abstract
Definition of the disease
Malignant atrophic papulosis (MAP), described independently by Köhlmeier and Degos et al., is a rare, chronic, thrombo-obliterative vasculopathy characterized by papular skin lesions with central porcelain-white atrophy and surrounding teleangiectatic rim.
Epidemiology
Less than 200 cases have been described in the literature. The first manifestation of MAP usually occurs between the 20th and 50th year of life.
Clinical description
The cutaneous clinical picture is almost pathognomonic.

2011

Degos Disease with Dermatomyositis-like Phenomenon: A Diagnostic Dilemma and a Therapeutic Challenge
Link: http://www.docguide.com/degos-disease-dermatomyositis-phenomenon-diagnostic-...
Authors: Gupta S, Dogra S, Saikia UN, Yadav S, Kanwar AJ
Reference: Journal of Cutaneous Medicine and Surgery 15 (3), 162-6 (2011 May-Jun)

Degos disease is a type of vasculopathy characterized by progressive occlusion of small-sized blood vessels primarily involving the skin, gastrointestinal system, and central nervous system as well as various other systems. Owing to the rarity of the condition, the diagnosis is often a challenge; consequently, management is even more difficult owing to the paucity of experience and literature for the effective treatment of this entity.

A man with generalized small white skin lesions and abdominal pain
Link: http://onlinelibrary.wiley.com/doi/10.1111/j.1365-4632.2010.04603.x/abstract
Authors: Tin Sik Cheng MB, MRCP, FHKCP, FHKAM (Medicine)
Department of Health, Social Hygiene Service,Centre for Health Protection, Hong Kong, China
Reference: Cheng, T. S. (2011), A man with generalized small white skin lesions and abdominal pain. International Journal of Dermatology, 50: 726–729. doi: 10.1111/j.1365-4632.2010.04603.x
Correspondence: Dr Tin Sik Cheng, Cheung Sha Wan, Dermatology Clinic, 3F West Kowloon Health Centre, 303 Cheung Sha Wan Road, Kowloon, Hong Kong, China <gtscheng@gmail.com>

International Journal of Dermatology Volume 50, Issue 6, pages 726–729, June 2011

First page of paper

Degos disease-like presentation in systemic lupus erythematosus

Link: http://www.ijdvl.com/text.asp?2011/77/2/219/77477
Authors: Najeeba Riyaz, Rahima Saleem, Roshni Shafeeq
Department of Dermatology and Venereology, Medical College, Calicut, India
Reference: DOI: 10.4103/0378-6323.77477

Degos disease (DD) or malignant atrophic papulosis or Kohlmeier-Degos-Delort-Tricort syndrome was first described in 1941 by Kohlmeier, and Robert Degos recognized it as a distinct entity in 1942. [1],2 This rare disorder is probably an endovasculitis affecting small- and medium-sized arteries leading to tissue infarction mainly in the skin, gastrointestinal system, and central nervous system. It is characterized by porcelain white atrophic papules with infarctive lesions. Variants of DD with only cutaneous involvement and association with collagen vascular disease have been described.

2010

Malignant Atrophic Papulosis: A Case Report with Severe Visual and Neurological Impairment
Link: http://onlinelibrary.wiley.com/doi/10.1111/j.1525-1470.2010.01287.x/abstract
Authors: Marta Gutiérrez-Pascual M.D.1, Angela Hernández-Martín M.D., Isabel Colmenero M.D., Juan José García-Peñas M.D., Miguel Angel López-Pino M.D., Antonio Torrelo M.D.
Department of Dermatology, Hospital Niño Jesús, Madrid, Spain
Reference: Pediatric Dermatology
Correspondence: Angela Hernández-Martín, M.D., Department of Dermatology, Hospital Infantil Niño Jesús Avda, Menéndez Pelayo, 65, 28009 Madrid, Spain. <ahernandez_hnj@yahoo.es>

Abstract:  Malignant atrophic papulosis, or Degos’ disease, is a severe systemic vasculopathy extremely rare in children. The skin, gastrointestinal tract, and central nervous system are most frequently affected. We report a 5-year-old girl with malignant atrophic papulosis who presented with widespread skin lesions from early infancy and severe visual and neurological impairment.

Post-streptococcal vasculopathy with evolution to Degos' disease
Link: http://www.sciencedirect.com/science?_ob=ArticleURL&_udi=B6T06-51BPDV6-2...
Authors: Sandipan Pati, a, , Suraj A. Muleya, Marie F. Grilla, Stephen Coonsa and Russell Walkera
Barrow Neurological Institute, 350 W Thomas Road, Phoenix, AZ 85013, United States
Reference:
Correspondence: Sandipan Pati, Corresponding author. Dept. of Neurology, 350 W Thomas Road, Phoenix, AZ 85013, United States. Tel.: + 1 480 652 3615. <sandipan.pati@chw.edu>

Degos’ disease or malignant atrophic papulosis is a rare disseminated occlusive vasculopathy affecting the skin, gastrointestinal tract, central nervous system, and less often other organ systems. The exact etiology of this vasculopathy has not been established. Infections, autoimmune disease and coagulation defects have been proposed as underlying pathogenic mechanisms, but none have been confirmed. Here, we report the clinical, radiological and histopathologic features of Degos’ disease in a 41-year-old man following streptococcal throat infection.

Infantile neurological Degos disease.
Link: http://www.ncbi.nlm.nih.gov/pubmed/20659809?dopt=Abstract
Authors: Yeo TH, Vassallo G, Judge M, Laycock N, Kelsey A, Crow YJ.
Department of Paediatric Neurology, Royal Manchester Children's Hospital, Manchester M13 9WL, UK.
Reference: Eur J Paediatr Neurol.

Degos disease, or malignant atrophic papulosis, is a rare vasculopathy of uncertain aetiology manifesting as a primary dermatological disorder in most cases, but with widespread systemic involvement developing in an undefined proportion of patients. Reported neurological features of Degos disease include ischaemic and haemorrhagic stroke, subdural effusion, seizures, neuropathy, transverse myelitis, and optic atrophy. The description of contrast enhancement of the leptomeninges possibly indicates a defect of blood vessel integrity likely explaining the pleiotropic neurological manifestations.

A case of Degos disease successfully treated with corticosteroid combined with cyclophosphamide
Link: http://www.degosdisease.com/Kameda_Degos Rx_RheumInt_10.pdf
Authors: Tomohiro Kameda, Hiroaki Dobashi, Kozo Yoneda, Kentaro Susaki, Toshiya Kuno, Koji Murao, Toshihiko Ishida
Department of Internal Medicine Division of Endocrinology and Metabolism, Hematology, Rheumatology and Respiratory Medicine Faculty of Medicine, Kagawa University, Takamatsu, Japan
Reference: Rheumatol Int DOI 10.1007/s00296-010-1436-z

2009

Pairing and comparing nine diseases with Degos Disease (Malignant Atrophic Papulosis): An attempt to illustrate our understanding and direct future inquiry
Link: http://dermatology-s10.cdlib.org/1501/commentary/degos/scheinfeld.html
Authors: Noah Scheinfeld MD JD
Department of Dermatology, Columbia University, New York, NY.
Reference: Dermatology Online Journal 15 (1): 10
Correspondence: Noah Scheinfeld <NSS32@columbia.edu>

2008

Painless pink papules with central porcelain-white scars
Link: http://www.cmaj.ca/cgi/content/full/179/11/1171
Authors: P. Régine Mydlarski, MD, Duane F. Barber, MD and Lynne H. Robertson, MD
Division of Dermatology, Department of Medicine, University of Calgary; Department of Pathology and Laboratory Medicine, University of Calgary, and Calgary Laboratory Services, Calgary, Alta
Reference: CMAJ, November 18, 2008; 179 (11)

2007

Malignant atrophic papulosis
Link: http://www.ingentaconnect.com/content/bsc/ced/2007/00000032/00000005/art00001
Authors: Scheinfeld, N
Reference: Clinical & Experimental Dermatology, Volume 32, Number 5, September 2007 , pp. 483-487(5)

A Case of Systemic Lupus Erythematosus with Degos' Disease
Link: http://www.koreamed.org/SearchBasic.php?RID=408953&DT=1&QY=degos+%5BALL...
Authors: Lee JH, Ryu HJ, Park YB, Choi BY, Lee EY, Lee YJ, Lee EB, Song YW
Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea
Reference: J Korean Rheum Assoc. 2007 Sep;14(3):256-262
Correspondence: Song YW <ysong@snu.ac.kr>

Malignant Atrophic Papulosis
Link: http://www.ispub.com/ostia/index.php?xmlFilePath=journals/ijd/vol5n2/papulosis....
Authors: Anuja Agarwal, K. N. Shivaswamy, Devinder M. Thappa, S. Jayanthi
Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry, India
Reference: The Internet Journal of Dermatology. 2007. Volume 5 Number 2.
Correspondence: Dr. Devinder Mohan Thappa <dmthappa@gmail.com>

2006

Degos' disease: Acute abdomen with skin rash
Link: http://www.indianjgastro.com/article.asp?issn=0254-8860;year=2006;volume=25;...
Authors: Rajesh R, Basu Adhish, Sistla Sarath Chandra, Jagdish S, Thappa Davinder Mohan, Badhe Bhawana Ashok
Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India
Reference: Indian J Gastroenterol 2006;25:187-187
Correspondence: Basu Adhish <adhishbasu@rediffmail.com>

Malignant atrophic papulosis: endocardial involvement and positive anticardiolipin antibodies
Link: http://www.blackwell-synergy.com/doi/abs/10.1111/j.1468-3083.2006.01339.x
Authors: E Saglik, C Baykal, N Buyukbabani, M Inanc
Department of Dermatology, Department of Pathology, and Division of Rheumatology, Department of Internal Medicine, Istanbul Medical Faculty, Istanbul University
Reference: Letter to Journal of the European Academy of Dermatology and Venereology 20 (5), 602-603
Correspondence: E Saglik <saglikmd@hotmail.com>

Malignant atrophic papulosis: endocardial involvement and positive anticardiolipin antibodies
Link: http://www.ingentaconnect.com/content/bsc/jdv/2006/00000020/00000005/art00020
Authors:
Reference: Journal of the European Academy of Dermatology & Venereology, Volume 20, Number 5, May 2006 , pp. 602-603(2)

Optic nerve and spinal cord manifestations of malignant atrophic papulosis (Degos disease)
Link: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&...
Authors: Matsuura F, Makino K, Fukushima T, Matsubara N, Shibuya M, Higuchi T, Hashidate H, Yamada M, Shibuya H, Yamazaki M
Department of Neurology, Niigata City General Hospital, 2-6-1 Shichikuyama, Niigata 950-8739, Japan
Reference: J Neurol Neurosurg Psychiatry. 2006 Feb;77(2):260-2
Correspondence: Dr Kunihiko Makino <makinoncgh@hosp.niigata.niigata.jp>

Optic nerve and spinal cord manifestations of malignant atrophic papulosis (Degos disease)
Link: http://www.medscape.com/medline/abstract/16421135?queryText=degos
Authors: F Matsuura, K Makino, T Fukushima, et. al.
Reference: J Neurol Neurosurg Psychiatry - Feb 2006

A fatal case of malignant atrophic papulosis (Degos disease) with optic nerve and spinal cord involvement is described

Optic nerve and spinal cord manifestations of malignant atrophic papulosis (Degos disease)
Link: http://cat.inist.fr/?aModele=afficheN&cpsidt=17450419
Authors: Matsuura F, Makino K, Fukushima T, Matsubara N, Shibuya M, Higuchi T, Hashidate H, Yamada M, Shibuya H, Yamazaki M
Niigata City General Hospital, Niigata, Japan; Pathological Neuroscience Branch, Brain Research Institute, Niigata University, Niigata, Japan
Reference: 2006, vol. 77, no2, pp. 260-262

A fatal case of malignant atrophic papulosis (degos disease)
Link: http://www.medscape.com/medline/abstract/16710585?queryText=degos
Authors: Thomas Hohwy, Martin Glümer Jensen, Anders Tøttrup, et. al.
Reference: Acta Derm Venereol - Jan 2006

Malignant atrophic papulosis (Degos’ disease) is a very rare condition characterized by atrophic papular skin lesions and variable association of systemic involvement

A fatal case of malignant atrophic papulosis (Degos' disease) in a man with factor V Leinden mutation and lupus anticoagulant.
Link: http://www.ncbi.nlm.nih.gov:80/pubmed/16710585
Authors: Hohwy T, Jensen MG, Tøttrup A, Steiniche T, Fogh K
Department of Dermatology, Aarhus University Hospital, Denmark
Reference: Acta Derm Venereol. 2006;86(3):245-7.
Correspondence: Thomas Hohwy <thomashohwy@hotmail.com>

2005

Degos disease associated with protein S deficiency
Link: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&...
Authors: Gilaberte Y, Coscojuela C, Lezaun A, Marigil MA
Reference: Br J Dermatol. 2005 Sep;153(3):666-7

Possible link between thrombophilia and Degos disease

Benign cutaneous Degos disease
Link: http://www.medscape.com/medline/abstract/16101866?queryText=degos
Authors: Mozheh Zamiri, Paul Jarrett
Reference: Int J Dermatol - Aug 2005

A 24-year-old woman presented with an 8-year history of a recurrent asymptomatic rash characterized by small erythematous papules which evolved to form atrophic porcelain white scars with a telangectatic rim.

Degos disease with an inconspicuous clinical course
Link: http://www.medscape.com/medline/abstract/15987298?queryText=degos
Authors: R Loewe, M Palatin
Reference: J Eur Acad Dermatol Venereol - Jul 2005

BACKGROUND: Degos disease is a rare systemic disorder with involvement of the skin and visceral organs, leading to death in about 50% of cases within 1 or 2 years

Painful penile ulceration in a patient with malignant atrophic papulosis
Link: http://www.blackwell-synergy.com/doi/abs/10.1111/j.1468-3083.2005.01227.x
Authors: K Aydogan, G Alkan, S Karadogan Koran, SB Adim, M Kiyici, N Tokgoz
Departments of Dermatology, Pathology and Gastroenterology, Uludag University School of Medicine, Bursa, Turkey
Reference: Journal of the European Academy of Dermatology & Venereology, Volume ? Issue ? - April 2005
Correspondence: K Aydogan <aydogank@uludag.edu.tr>

Nervous System Involvement in Degos' Disease
Link: http://www.ajnr.org/cgi/content/abstract/26/3/646?maxtoshow=&HITS=10&...
Authors: Carmelo Amato, Raffaele Ferri, Maurizio Elia, Filomena Cosentino, Carmelo Schepis, Maddalena Siragusa and Massimo Moschini
Reference: American Journal of Neuroradiology 26:646-649, March 2005

A case of Systemic Malignant Atrophic Papulosis (Köhlmeier-Degos' Disease)
Link: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&...
Authors: Evans R. Fernandez-Perez MD, Erica Grabscheid MD and Noah S. Scheinfeld MD, JD, FAAD
Reference: Journal of the National Medical Association, Vol. 97, No. 3, March 2005

Lesions resembling malignant atrophic papulosis in a patient with progressive systemic sclerosis
Link: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&...
Authors: Liu CM, Harris RM, Hansen CD
Reference: Cutis. 2005 Feb;75(2):101-4.

Degos' disease is probably a distinct entity: A review of clinical and laboratory evidence
Link: http://www.sciencedirect.com/science?_ob=ArticleURL&_udi=B6WM8-4FC365G-1P...
Authors: Noah Scheinfeld
Department of Dermatology, St. Luke's Roosevelt Hospital Center, New York, New York
Reference: Journal of the American Academy of Dermatology Volume 52, Issue 2, February 2005, Pages 375-376

2004

Is Degos' Disease a clinical and histological end point rather than a specific disease?
Link: http://www.degosdisease.com/info/AmAcadDermatol_2004_06.pdf
Authors: High WA, Aranda J, Patel SB, Cockerell CJ, Costner MI.
Reference: J Am Acad Dermatol. 2004 Jun;50(6):895-9.
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