Degos Disease Treatment & Management

  • Author: Noah S Scheinfeld, MD, JD, FAAD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Aug 2, 2011
 

Medical Care

Until 2010 no medical therapy for Degos disease (DD) was ntoed to be uniformly effective. Antiplatelet drugs (eg, aspirin, dipyridamole) may reduce the number of new lesions in some patients with only skin involvement. Some believe intravenous immunoglobulin may have a role in treatment.[35] This has changed with the reports that eculizamab can effectively treat systemic Degos Disease.[1]

Other treatments that have been tried without real effect include the following: topical corticosteroids, phenformin and ethylestrenol, iodohydroxyquinoline, aspirin and dipyridamole, phenylbutazone, arsenic, sulphonamides, dextran, corticosteroids, heparin, warfarin, niacin, streptomycin, corticotropin, azathioprine, methotrexate, cyclosporine, tacrolimus, mycophenolate mofetil, pentoxifylline, and clopidogrel.

A presentation by Magro et al[36] reported that eculizumab, which has been approved for paroxysmal nocturnal hemoglobinuria, improved systemic Degos disease in 3 patients. This small series was uncontrolled, but the results suggest promise for eculizumab as a treatment. The effectiveness of eculizumab also may suggest that Degos disease possibly is not a collagen-vascular disease, but instead is an endothelial- or complement-mediated disease. Eculizumab is expensive, which may hinder its acceptance as widely used therapy.

Hohwy et al[31] treated a patient with fatal systemic malignant atrophic papulosis (MAP) with narrow-band ultraviolet (UVB) light and prednisolone and, later, with aspirin, pentoxifylline, and warfarin. Treatment was not effective. Interestingly, the patient has a mutation of factor V Leiden and lupus anticoagulant.

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Surgical Care

When gastrointestinal bleeding, intestinal perforation, bowel infarction, or intracranial bleeding occurs, proper surgical intervention is necessary.

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Consultations

Consult the following physicians:

  • Dermatologists
  • Gastroenterologists
  • Neurologists
  • General surgeons
  • Neurosurgeons
  • Ophthalmologists
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Activity

Patients are often weak and this limits their activity, but no limitation to Degos disease itself exists.

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Contributor Information and Disclosures
Author

Noah S Scheinfeld, MD, JD, FAAD  Assistant Clinical Professor, Department of Dermatology, Columbia University College of Physicians and Surgeons; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, and New York Eye and Ear Infirmary; Private Practice

Noah S Scheinfeld, MD, JD, FAAD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Optigenex Consulting fee Independent contractor

Specialty Editor Board

Franklin Flowers, MD  Chief, Division of Dermatology, Professor, Department of Medicine and Otolaryngology, Affiliate Associate Professor of Pediatrics and Pathology, University of Florida College of Medicine

Franklin Flowers, MD, is a member of the following medical societies: American College of Mohs Micrographic Surgery and Cutaneous Oncology

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Jeffrey J Miller, MD  Associate Professor of Dermatology, Pennsylvania State University College of Medicine; Staff Dermatologist, Pennsylvania State Milton S Hershey Medical Center

Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Professors of Dermatology, North American Hair Research Society, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

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  2. Scheinfeld N. Pairing and comparing nine diseases with Degos Disease (Malignant Atrophic Papulosis): an attempt to illustrate our understanding and direct future inquiry. Dermatol Online J. Jan 15 2009;15(1):10. [Medline].

  3. High WA, Aranda J, Patel SB, Cockerell CJ, Costner MI. Is Degos' disease a clinical and histological end point rather than a specific disease?. J Am Acad Dermatol. Jun 2004;50(6):895-9. [Medline].

  4. Ball E, Newburger A, Ackerman AB. Degos' disease: a distinctive pattern of disease, chiefly of lupus erythematosus, and not a specific disease per se. Am J Dermatopathol. Aug 2003;25(4):308-20. [Medline].

  5. Scheinfeld N. Degos' disease is probably a distinct entity: a review of clinical and laboratory evidence. J Am Acad Dermatol. Feb 2005;52(2):375-6; author reply 376-8. [Medline].

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  14. Lankisch MR, Johst P, Scolapio JS, Fleming CR. Acute abdominal pain as a leading symptom for Degos' disease (malignant atrophic papulosis). Am J Gastroenterol. Apr 1999;94(4):1098-9. [Medline].

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  22. Fernandez-Flores A, Manjon JA, Mencia J. Diabetes mellitus and Degos disease. Bratisl Lek Listy. 2008;109(9):414-7. [Medline].

  23. Dyrsen ME, Iwenofu OH, Nuovo G, Magro CM. Parvovirus B19-associated catastrophic endothelialitis with a Degos-like presentation. J Cutan Pathol. Oct 2008;35 Suppl 1:20-5. [Medline].

  24. Bogenrieder T, Kuske M, Landthaler M, Stolz W. Benign Degos' disease developing during pregnancy and followed for 10 years. Acta Derm Venereol. 2002;82(4):284-7. [Medline].

  25. Pati S, Muley SA, Grill MF, Coons S, Walker R. Post-streptococcal vasculopathy with evolution to Degos' disease. J Neurol Sci. Jan 15 2011;300(1-2):157-9. [Medline].

  26. Ortiz A, Ceccato F, Albertengo A, Roverano S, Iribas J, Paira S. Degos cutaneous disease with features of connective tissue disease. J Clin Rheumatol. Apr 2010;16(3):132-4. [Medline].

  27. Tan WP, Chio MT, Ng SK. Generalized red papules with gastrointestinal complications. Diagnosis: malignant atrophic papulosis (Degos' disease). Clin Exp Dermatol. Sep 2007;32(5):615-6. [Medline].

  28. Egan R, Lessell S. Posterior subcapsular cataract in Degos disease. Am J Ophthalmol. Jun 2000;129(6):806-7. [Medline].

  29. Matsuura F, Makino K, Fukushima T, et al. Optic nerve and spinal cord manifestations of malignant atrophic papulosis (Degos disease). J Neurol Neurosurg Psychiatry. Feb 2006;77(2):260-2. [Medline].

  30. Sibillat M, Avril MF, Charpentier P, Offret H, Bloch-Michel E. [Malignant atrophic papulosis (Degos' disease): clinical review. Apropos of a case]. J Fr Ophtalmol. 1986;9(4):299-304. [Medline].

  31. Hohwy T, Jensen MG, Tottrup A, Steiniche T, Fogh K. A fatal case of malignant atrophic papulosis (Degos' disease) in a man with factor V Leinden mutation and lupus anticoagulant. Acta Derm Venereol. 2006;86(3):245-7. [Medline].

  32. Yoshikawa H, Maruta T, Yokoji H, Takamori M, Yachie A, Torii Y. Degos' disease: radiological and immunological aspects. Acta Neurol Scand. Nov 1996;94(5):353-6. [Medline].

  33. Amaravadi RR, Tran TM, Altman R, Scheirey CD. Small bowel infarcts in Degos disease. Abdom Imaging. Mar-Apr 2008;33(2):196-9. [Medline].

  34. Scheinfeld N. Malignant atrophic papulosis. Clin Exp Dermatol. Sep 2007;32(5):483-7. [Medline].

  35. Zhu KJ, Zhou Q, Lin AH, Lu ZM, Cheng H. The use of intravenous immunoglobulin in cutaneous and recurrent perforating intestinal Degos disease (malignant atrophic papulosis). Br J Dermatol. Jul 2007;157(1):206-7. [Medline].

  36. Magro C, Shapiro L, Johnson B, Salmon J, Whelan P. Rheumatology Grand Rounds Hospital for Special Surgery (New York City) Degos Disease. Feb 10, 2010. In press.

  37. Beuran M, Chiotoroiu AL, Morteanu S, Chilie A, Avram M, Rosu O, et al. Köhlmeier-Degos disease (Malignant Atrophic Papulosis): a cause of recurrent multiple intestinal perforations. Chirurgia (Bucur). Nov-Dec 2009;6:765-72. [Medline].

 
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Four papules of Degos disease located on upper-inner arm in different stages of evolution. Courtesy of David F. Butler, MD.
Papule of Degos disease with central sclerosis and telangiectasia. Courtesy of David F. Butler, MD.
Superficial and mid-dermal perivascular lymphocytic infiltrate with focal vacuolar change at the dermoepidermal junction (hematoxylin and eosin, X100). Courtesy of David F. Butler, MD.
Alcian blue stain for mucin (X100). Courtesy of David F. Butler, MD.
Wedge-shaped area of dermal sclerosis (hematoxylin and eosin, X100). Courtesy of David F. Butler, MD.
 
 
 
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