eMedicine Specialties > Dermatology > Diseases of the Vessels

Degos Disease: Differential Diagnoses & Workup

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

Updated: Dec 21, 2009

Differential Diagnoses

Lupus Erythematosus, Acute

Other Problems to Be Considered

Primary ulceration of the small intestine
Crohn Disease
Atrophie blanche lesions in systemic lupus erythematosus
Dermal mucinosis
Wegener granulomatosis

Workup

Laboratory Studies

  • No specific laboratory test can be used to aid in diagnosing Degos disease (DD). In fact, most laboratory test results are normal, with the exception of the manifestation of anemia secondary to intestinal bleeding.
  • In 1 patient, laboratory examinations disclosed persistent elevations of the thrombin-antithrombin III complex, plasmin-alpha 2 plasmin inhibitor complex, and cytotoxic T-cell subset (CD8+ CD11-), illustrating the coagulative, fibrinolytic, and immunologic implications of Degos disease.
  • In a series of 3 patients with Degos disease, prolonged euglobulin lysis time, increased plasminogen activator levels, and increased plasminogen activator inhibitor activities were detected before and after a venous occlusion test, indicating an inhibition of fibrinolysis.
  • In a series of 3 patients with Degos disease, electron microscopy demonstrated an increased number of Weibel-Palade bodies and a raised staining of von Willebrand factor in endothelial cells in 1 patient.
  • In a series of 3 patients, test results for coagulation and circulating anticoagulant were in the reference range.
  • In a series of 3 patients, the results of platelet adhesion showed decreased adhesion in 1 patient and increased adhesion in another patient. Platelet aggregation study results decreased to the reference range in 2 patients. In one patient, tests showed hyperactive spontaneous and induced platelet aggregation.
  • Amounts of protein in the cerebrospinal fluid and amounts of platelet aggregation have been reported as sharply increased in progressive stages of the disease. For example, laboratory results showed a gradual increase of cerebrospinal fluid proteins (from 156 mg/dL to 602 mg/dL) and an extremely increased amount of platelet aggregation.
  • In Degos disease, viruslike inclusions are frequently present in the endothelial cells and fibroblasts. C3 deposits and the presence of intracytoplasmic cylinders are also frequently present in the histiocytes.
  • Hohwy et al28 reported a fatal case of systemic malignant atrophic papulosis (MAP) in a man with factor V Leiden mutation and lupus anticoagulant.

Imaging Studies

  • MRI of the brain can show multiple cerebral infarctions accompanied by small hemorrhagic areas and gadolinium-diethylenetriamine pentaacetic acid enhancement of the dura.29 They can also reveal intracerebral bleeding, subdural hemorrhage, and cord infarcts.
  • A cerebral angiogram may reveal narrowing and occlusion of small intracranial arteries. Specifically, a cerebral angiogram can depict stenosis, ectasia, and aneurysms involving the peripheral branch of arteries.29
  • EEG tests have shown generalized nonspecific slowing.
  • In some patients, electromyograms demonstrate axonal and demyelinating polyneuropathy.
  • With angiograms, stenoses can be observed in the celiac artery and the small arteries in the kidney.
  • In some patients, chest radiographs have depicted extensive calcification of the pericardium. Although pleural involvement and pericardial involvement have been reported in Degos disease, constrictive pericarditis is most unusual, and radiographically demonstrable calcification of the pericardium has not been previously reported.
  • Matsuura et al26 noted a fatal case of malignant atrophic papulosis involving the optic nerve and spinal cord. MRI of the optic nerve demonstrated (1) pathologic signal enhancement on fat-suppressed, T1-weighted images after intravenous meglumine gadopentetate infusion and (2) a sawtooth pattern over 7 vertebral segments of the spinal cord on T2-weighted sagittal images. These findings were confirmed at autopsy, during which pathologists grossly noted (1) a pronounced decrease of myelinated nerve fibers in the left optic nerve with thrombotic obstruction of the central retinal artery, (2) spongy degeneration in all levels of the spinal cord, and (3) patchy and moth-eaten patterns due to thromboses and endothelial proliferation in subarachnoid vessels.
  • Amaravadi et al30 described CT scan findings a 40-year-old woman. CT scanning of the abdomen and pelvis with oral and intravenous contrast demonstrated extensive ascites and nodular thickening of the omentum. A subsequent CT scan of the abdomen and pelvis with intravenous contrast showed a large amount of ascites with small bowel wall thickening and patchy mucosal perfusion and intraluminal hemorrhage into a loop of infarcted jejunum, which was later confirmed at autopsy to be an intraluminal clot. A final CT scan of the abdomen and pelvis with only intravenous contrast, which was performed to investigate worsening peritonitis, showed extensive pathology. An axial image demonstrated pneumoperitoneum with edematous and hyperemic small bowel loops consistent with small bowel ischemia. Discontinuity in the wall of a loop of jejunum was consistent with perforation. Multiple jejunal perforations were later demonstrated at autopsy.

Other Tests

  • Endoscopy of the gastrointestinal tract (ie, stomach, esophagus, duodenum, colon, rectum) can show infarcted lesions or ulcers.
  • Laparoscopy of the intestine can show similar type lesions that manifest with white plaques with red borders on the serosal surface of the bowel and the peritoneum.
  • In 2007, Amaravadi et al30 reported autopsy findings from a 40-year-old female Degos disease patient. They found omental infarction and multiple yellow plaques on the serosal surface of the small bowel during an exploratory laparotomy.

Histologic Findings

Early papules in Degos disease are skin colored and can demonstrate a superficial and deep perivascular, periadnexal, and perineural chronic inflammatory cell infiltrate associated with interstitial mucin deposition, as shown in the images below.

Superficial and mid-dermal perivascular lymphocyt...

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.

[ CLOSE WINDOW ]
Superficial and mid-dermal perivascular lymphocyt...

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 D...

Alcian blue stain for mucin (X100). Courtesy of David F. Butler, MD.

[ CLOSE WINDOW ]
Alcian blue stain for mucin (X100). Courtesy of D...

Alcian blue stain for mucin (X100). Courtesy of David F. Butler, MD.


A mild vacuolar interface reaction has been described. The vacuolar interface dermatitis, dermal mucin, and perivascular lymphoid infiltrate mimic lupus erythematosus.

Fully developed papules can be raised, with umbilicated porcelain-white centers and a surrounding erythematous rim. Histologically, these papules demonstrate wedge-shaped degeneration of collagen, as shown in the image below.
 

Wedge-shaped area of dermal sclerosis (hematoxyli...

Wedge-shaped area of dermal sclerosis (hematoxylin and eosin, X100). Courtesy of David F. Butler, MD.

[ CLOSE WINDOW ]
Wedge-shaped area of dermal sclerosis (hematoxyli...

Wedge-shaped area of dermal sclerosis (hematoxylin and eosin, X100). Courtesy of David F. Butler, MD.


An interface dermatitis can be present but is often limited to the central portion of the tissue examined histologically. Additionally, squamatization of the dermoepidermal junction, melanin incontinence, and epidermal atrophy can manifest.31

In many cases, an area of papillary dermal sclerosis manifests that mirrors the early stages of lichen sclerosus et atrophicus.

Hyperkeratosis, epidermal atrophy, dermoepidermal separation, edema, and papillary dermal necrosis occur. Fibrinoid necrosis and thrombosis occur in the papillary dermis and in the capillary and venules. Others have noted that skin biopsy specimens show hyperkeratosis, atrophy of the epidermis, and necrobiosis of the collagen layer. Well-developed papules of Degos disease with epidermal atrophy and hyperkeratosis overlying a wedge-shaped area of cutaneous ischemia extending into the deep dermis have also been observed.

A superficial and deep perivascular lymphocytic infiltrate can gather at the fringe of ischemic areas. Marked endothelial swelling and occasional platelet-fibrin thrombi are often noted. Infarctive changes or scattered necrotic keratinocytes may be present in the epidermis. Abundant acid mucopolysaccharides often occur in the dermis, mimicking dermal mucinosis.
Direct immunofluorescence examination does not yield definitive results. Perivascular fibrin and complement can be present. In one reported case, autopsy results demonstrated the clinical diagnosis of Degos disease based on the finding of thromboangiitis of the Burger type, with bland infarcts of the small intestine and perforation of the jejunum.

Microaneurysms of the bulbar conjunctival vessels have been described.

Changes in the kidneys manifest with thickening of the afferent glomerular arterioles and the capillary basement membrane.

In 2008, Notash et al8 described a fatal case of Degos disease in a 48-year-old man whose autopsy findings demonstrated diffuse fibrotic changes in the serosal membranes and the internal organs.

More on Degos Disease

Overview: Degos Disease
Differential Diagnoses & Workup: Degos Disease
Treatment & Medication: Degos Disease
Follow-up: Degos Disease
Multimedia: Degos Disease
References
[ CLOSE WINDOW ]

References

  1. 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].

  2. 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].

  3. 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].

  4. 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].

  5. Wilson J, Walling HW, Stone MS. Benign cutaneous Degos disease in a 16-year-old girl. Pediatr Dermatol. Jan-Feb 2007;24(1):18-24. [Medline].

  6. Guhl G, Diaz-Ley B, Delgado Y, Dauden E, Fraga J, Garcia-Diez A. Wegener's granulomatosis: a new entity in the growing differential diagnosis of Degos' disease. Clin Exp Dermatol. Jul 2009;34(5):e1-3. [Medline].

  7. Subbiah P, Wijdicks E, Muenter M, Carter J, Connolly S. Skin lesion with a fatal neurologic outcome (Degos' disease). Neurology. Mar 1996;46(3):636-40. [Medline].

  8. Notash AY, Mazoochy H, Mirshams M, Nikoo A. Lethal systemic Degos disease with prominent cardio-pulmonary involvement. Saudi Med J. Jan 2008;29(1):133-7. [Medline].

  9. Rajesh R, Basu A, Sistla SC, Jagdish S, Thappa DM, Badhe BA. Degos' disease: acute abdomen with skin rash. Indian J Gastroenterol. Jul-Aug 2006;25(4):187. [Medline].

  10. Kim DW, Kang SB, Lee KH, Choe GY, Park SY, Nicholay M. Degos' disease (malignant atrophic papulosis) as a fatal cause of acute abdomen: report of a case. Surg Today. 2008;38(9):866-70. [Medline].

  11. al-Smadi RM, Abu-Jamous F, Omeish I. Degos disease in a 24-year-old Jordanian male. East Mediterr Health J. Jan 2000;6(1):194-6. [Medline].

  12. Katz SK, Mudd LJ, Roenigk HH Jr. Malignant atrophic papulosis (Degos' disease) involving three generations of a family. J Am Acad Dermatol. Sep 1997;37(3 Pt 1):480-4. [Medline].

  13. 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].

  14. Jalil J, Shafique M, Rashid Dar N. Dermatological clue to diagnosis of Degos disease in a 2-year-old with obscure chronic abdominal pain. Clin Pediatr (Phila). Mar 2008;47(2):180-2. [Medline].

  15. Farrell AM, Moss J, Costello C, Fearfield LA, Woodrow D, Bunker CB. Benign cutaneous Degos' disease. Br J Dermatol. Oct 1998;139(4):708-12. [Medline].

  16. Requena L, Farina C, Barat A. Degos disease in a patient with acquired immunodeficiency syndrome. J Am Acad Dermatol. May 1998;38(5 Pt 2):852-6. [Medline].

  17. Torrelo A, Sevilla J, Mediero IG, Candelas D, Zambrano A. Malignant atrophic papulosis in an infant. Br J Dermatol. May 2002;146(5):916-8. [Medline].

  18. Salomon MI, Mandel EH, Gallo G. Degos' disease associated with a "spontaneous cure" of diabetes. J Am Geriatr Soc. Nov 1971;19(11):923-32. [Medline].

  19. Asherson RA, Cervera R. Antiphospholipid syndrome. J Invest Dermatol. Jan 1993;100(1):21S-27S. [Medline].

  20. Saglik E, Baykal C, Buyukbabani N, Inanc M. Malignant atrophic papulosis: endocardial involvement and positive anticardiolipin antibodies. J Eur Acad Dermatol Venereol. May 2006;20(5):602-3. [Medline].

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

  22. 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].

  23. 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].

  24. 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].

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

  26. 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].

  27. 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].

  28. 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].

  29. 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].

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

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

  32. 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].

  33. Chung HY, Trendell-Smith NJ, Yeung CK, Mok MY. Degos' disease: a rare condition simulating rheumatic diseases. Clin Rheumatol. Jul 2009;28(7):861-3. [Medline].

  34. Coskun B, Saral Y, Cicek D, Ozercan R. Benign cutaneous Degos' disease: a case report and review of the literature. J Dermatol. Aug 2004;31(8):666-70. [Medline].

  35. Harvell JD, Williford PL, White WL. Benign cutaneous Degos' disease: a case report with emphasis on histopathology as papules chronologically evolve. Am J Dermatopathol. Apr 2001;23(2):116-23. [Medline].

  36. Pinault AL, Barbaud A, Weber-Muller F, Schmutz JL. [Benign familial Degos disease]. Ann Dermatol Venereol. Nov 2004;131(11):989-93. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

Degos disease, DD, malignant atrophic papulosis, papulosis atrophicans maligna, Kohlmeier-Degos-Delort-Tricot syndrome, Kohlmeier-Degos syndrome, Köhlmeier-Degos' disease, Online Mendelian Inheritance in Man 602248, OMIM 602248, papuleuse maligne atrophiante, lethal cutaneous and gastrointestinal arteriolar thrombosis, fatal cutaneointestinal syndrome, thromboangiitis cutaneointestinalis disseminata, dermatite papulosquameuse atrophiante, MAP

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Noah S Scheinfeld, MD, JD, FAAD, Assistant Clinical Professor, Department of Dermatology, Columbia University; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, 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

Medical Editor

Franklin Flowers, MD, Chief, Division of Dermatology, Professor, Department of Medicine and Otolaryngology, 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.

Pharmacy Editor

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.

Managing Editor

Jeffrey J Miller, MD, Associate Professor of Dermatology, Penn State University College of Medicine; Staff Dermatologist, Penn 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.

CME Editor

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, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.