Articles & Papers – 3

2008

Degos’ disease (malignant atrophic papulosis) as a fatal cause of acute abdomen: report of a case.

Link: http://www.ncbi.nlm.nih.gov/pubmed/18751957

Authors: Kim DW1, Kang SB, Lee KH, Choe GY, Park SY, Nicholay M.

1Department of Surgery, Seoul National University Bundang Hospital, 300 Gumi-dong, Bundang-gu, 463-707, Seongnam, Republic of Korea.

Reference:

Degos’ disease, otherwise known as “malignant atrophic papulosis,” is a rare condition characterized by typical cutaneous lesions. Its involvement of the gastrointestinal (GI) tract is usually associated with a poor prognosis. We report a case of Degos’ disease with GI involvement, which ultimately caused peritonitis, sepsis, and death, despite all treatment measures. A 59-year old woman was admitted to our hospital with acute generalized abdominal pain.

Inefficacy of intravenous immunoglobulins and infliximab in Degos’ disease.

Link: http://www.ncbi.nlm.nih.gov/pubmed/18575050

Authors: De Breucker S1, Vandergheynst F, Decaux G.

Reference: Acta Clin Belg. 2008 Mar-Apr;63(2):99-102.

We report the case of a 60-year-old man who presented with sudden visual loss, a history of postprandial abdominal pain, malabsorption, and skin lesions typical of systemic Degos’ disease. Despite anti-aggregants and prednisone the patient’s status did not improve. On the basis of the hypothetical dysimmune origin of this disease, we attempted treatment with intravenous immunoglobulins, without success. We then administered infliximab (Remicade), but 2 months after the third injection the patient developed mesenteric infarction and died.

Degos disease (Malignant atrophic papulosis) as a fatal cause of acute abdomen: report of a case

Link:

Authors: Kim Duck-Woo, Kang Sung-Bum et al

Reference: Surgery Today 2008; 38:866-870

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>

Generalized red papules with gastrointestinal complications. Diagnosis: malignant atrophic papulosis (Degos’ disease).

Link: http://www.ncbi.nlm.nih.gov/pubmed/17376207

Authors: Tan WP1, Chio MT, Ng SK.

Dept of Dermatology, National Skin Centre, Singapore.

Reference: Clin Exp Dermatol. 2007 Sep;32(5):615-6. Epub 2007 Mar 21.

Correspondence: <melissatanweeping@yahoo.com.sg>

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>

The use of intravenous immunoglobulin in cutaneous and recurrent perforating intestinal Degos disease (malignant atrophic papulosis)

Link: http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2133.2007.07951.x/full

Authors: K.J. Zhu, Q. Zhou, A.H. Lin, Z.M. Lu andH. Cheng

Reference: British Journal of Dermatology Volume 157, Issue 1, pages 206–207, July 2007

No abstract available for this article.

Benign cutaneous Degos disease in a 16-year-old girl

Link: http://www.ncbi.nlm.nih.gov/pubmed/17300643

Authors: Wilson J1, Walling HW, Stone MS.

Reference: Pediatr Dermatol. 2007 Jan-Feb;24(1):18-24.

Degos disease is a rare disorder, characterized by the development of typical atrophic porcelain-white macules on the trunk and extremities. It most commonly occurs in middle-aged Caucasian adults and eventuates in fatality in the majority of patients. A minority of reported patients have a more benign course and a favorable outcome. Degos disease is extremely rare in children. We report Degos disease in a teenager with an extended survival and the absence of internal involvement.

2006

Degos’ disease and abnormal MRI

Link: http://strokenotes.blogspot.com/2007/01/degos-disease-and-abnormal-mri.html

Authors: Neurodoc

Reference: NEJM Case 38-2006 355:24 Dec 14, 2006 p.2575

Correspondence: <Djacobs272@aol.com>

Stroke Notes
Notes from stroke reading cerebrovascular disease acute stroke blog. These are notes to myself for my own benefit but anyone may use

Synopsis: A 5 yo boy developed HA, increased abdominal girth and a rash. Exam showed pappilledema. MRI showed subdural hygromas.The hygromas were bilateral and highly proteinaceous. He also had projectile vomiting. The HA were early day variety, relieved with tylenol and ppt by exercise. The rash was centripetal maculopapular. There were crops of papules on the trunk, arms and legs, sparing palms and soles,and face.

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>

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

A Fatal Case of Malignant Atrophic Papulosis (Degos’ Disease) in a Man with Factor V Leiden Mutation and Lupus Anticoagulant

Link: http://www.medicaljournals.se/acta/content/?doi=10.2340/00015555-0068&html=1

Authors: Thomas Hohwy1, Martin Glümer Jensen2, Anders Tøttrup2, Torben Steiniche3 and Karsten Fogh1

1Department of Dermatology, 2Surgery and 3Pathology, Aarhus University Hospital, Denmark

Reference: Acta Derm Venereol 2006; 86: 245–247.

Correspondence: Thomas Hohwy, Department of Dermatology, Aarhus University Hospital, P. P. Oerumsgade 11, DK-8000 Aarhus C, Denmark < thomashohwy@hotmail.com>

Malignant atrophic papulosis (Degos’ disease) is an extremely rare disease with only approximately 150 cases reported in the international literature. Köhlmeier (1) reported the first case in 1941 and a year later Degos et al. (2) reported another case as a distinctive entity. It is a frequently fatal occlusive vasculopathy of unknown aetiology. It affects mainly the skin, gastrointestinal tract and central nervous system. There is a 3:1 male predominance (3) and onset is usually in the second through fourth decade (4).

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.org/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.

Inflammatory linear vasculopathy mimicking Degos’ disease

Link: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&…

Authors: Kirkup ME, Hunt SJ, Pawade J, Kennedy CT

Departments of Dermatology and Histopathology, Bristol Royal Infirmary, Bristol, UK

Reference: Br J Dermatol. 2004 Jun;150(6):1225-7

2003

Degos’ Disease: a distinctive pattern of disease, chiefly of lupus erythematosus, and not a specific disease per se.

Link: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&…

Authors: Ball E, Newburger A, Ackerman AB

Reference: Am J Dermatopathol, 25(4):308-20, Aug 2003

Benign course of malignant atrophic papulosis (Kohlmeier-Degos disease): lack of vessel occlusion as good prognostic sign?

Link: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&…

Authors: Wachter T, Rose C, Brocker EB, Leverkus M

Reference: J Dtsch Dermatol Ges. 2003 May;1(5):374-7

Malignant atrophic papulosis. Report of a case with multiple ophthalmic findings.

Link: http://www.ijo.in/article.asp?issn=0301-4738;year=2003;volume=51;issue=3;spage=…

Authors: Thomas Reji K, Nithyanandam S, Rawoof B Abdul, Rajendran SC

Department of Ophthalmology, St. John’s National Academy of Health Sciences, Johnnagar, Bangalore, India

Reference: Indian Journal of Opthalmology, Vol. 51, Iss. 3, pp. 260-3, 2003

Presentation of Degos Syndrome as Acute Small-Bowel Perforation

Link: http://archsurg.ama-assn.org/cgi/content/abstract/138/1/57

Authors: Drs González Valverde, Menarguez Pina, Mauri Barbera, Luri Prieto, García Marcilla, Vázquez Rojas, Ruiz, Gómez Ramos

Hospital General Vega Baja, Servicio de Cirugía General, Murcia, Spain

Reference: Archives of Surgery, Vol. 138, No. 1, January 2003