Degos Disease Clinical Presentation

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

History

In most cases, the first manifestation of Degos disease is a rash. Some have stated that the disease involves the skin as the sole clinical manifestation in 37% of patients. The gastrointestinal tract is involved in about 50% of patients. Additionally, note the following:

  • Patients may have a family history of Degos disease. In 1997, Katz et al[13] described a familial variant of Degos disease.
  • Degos disease associated with a spontaneous cure of diabetes has been reported.[19]
  • Patients with Degos disease may have a history of a variety of abdominal complaints, manifesting with abdominal pain, abdominal distention, cramps, nausea, vomiting, diarrhea, or constipation. Sometimes, patients may state that they experience weakness, fatigue, or weight loss, or symptoms of malabsorption.
  • Degos disease has been associated with systemic diseases. Degos disease has occurred in patients with rheumatoid arthritis, HIV infection, and antiphospholipid antibodies and antiphospholipid syndrome.[20]
  • Patients may have a history of a variety of neurologic complaints that include facial and acral paresthesia, headaches, dizziness, seizures, hemiplegia, aphasia, paraplegia, and gaze palsy.
  • Patients may have a variety of eye problems, including diplopia, ptosis, and visual-field defects.
  • Patients may experience weakness, shortness of breath, and chest pain.
  • Inflammatory linear vasculopathy mimicking Degos disease has been described. This is important to note because Degos disease mimicking vasculitis has been described.
  • Saglik et al[21] noted a case of malignant atrophic papulosis with endocardial involvement and positive anticardiolipin antibodies.
  • A case of Degos disease occurring with diabetes mellitus, with biopsy findings of both Degos disease and hyaline diabetic microangiopathy, has been reported.[22]
  • Parvovirus B19–associated catastrophic endothelialitis with a Degos-like presentation has been noted.[23]
  • The benign variant developing during pregnancy has been described.[24]
  • Poststreptococcal vasculopathy that evolved into Degos disease – like white papules has been reported.[25]
  • Ortiz et al reported a case of cutaneous Degos disease that showed features of connective-tissue disease; a specific connective-tissue disease never developed in the patient.[26]
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Physical

The main physical finding of Degos disease is a skin rash that manifests with porcelain white scars. Degos disease also affects the eyes, the intestines, the brain, and other organs with a variety of physical findings. Note the following physical findings.

  • The most visible manifestation of Degos disease is in the skin.
  • Degos disease manifests with (in most cases) multiple, small, red, raised papules that are 2-5 mm in diameter. After a few days, they enlarge and develop a central white spot that is depressed in comparison to the red skin around it, as shown in the images below. They heal, leaving depressed porcelain white scars with a rim of telangiectases. Note the images below. Four papules of Degos disease located on upper-innFour 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 andPapule of Degos disease with central sclerosis and telangiectasia. Courtesy of David F. Butler, MD.
  • The papules predominantly occur on the trunk and the arms.
  • Degos papules commonly manifest on the penis.[9]
  • Degos more rarely occurs on the palms and soles.[9]
  • One case has noted that Degos occurred on the scalp.
  • No cases known to the author note Degos disease-type papules on the face.
  • In a 17-month-old child, progressive involvement of the fingers and the toes with torpid ulcers and apical necrotic amputations has been reported.
  • Peristomal lesions have been reported.
  • The rash of Degos disease develops slowly and is usually mostly asymptomatic, but it may be accompanied by a slight burning sensation. The rash can arise anywhere except on the soles, the palms, and the face. The rash starts as pink or red papules that are 2-15 mm in diameter. The papules evolve into atrophic scars that are porcelain white. Sometimes, abdominal symptoms precede the rash, but this finding is uncommon.[27]
  • A variety of ocular findings occur in Degos disease. Posterior subcapsular cataracts,[28] visual field defects, ptosis, third cranial nerve palsies, blepharoptosis, and optic atrophy may be associated with Degos disease. Optical neuritis, papilledema, and scleral plaques can be present.[29] In 1986, Sibillat et al[30] reported that ophthalmologic symptoms were present in 35 of 105 extant reports of Degos disease. Degos disease manifested in the eye tissues, usually in the conjunctiva. The sclera, the episclera, the retina, the choroid, the optic nerve, and/or the neuro-ophthalmologic apparatus demonstrated damage consonant with Degos disease.
  • Constrictive pericarditis has been reported in Degos disease. This condition might be induced by pericardial vasculitis, thereby causing the left ventricular wall motion abnormality.
  • The lungs can be affected in Degos disease. Pulmonary manifestations include pleuritis and bilateral pleural effusions.
  • The liver and the kidneys may be involved and associated with a vasculitis.
  • The brain and the nerves can be affected in Degos disease. In 1 patient with Degos disease, the neurologic examination revealed a right-hemianopsia, paraparesis (with a sensory level at Th12), and a neurogenic bladder. In another patient, an ascending thoracic myelopathy was present. In 1996, at the Mayo Clinic, Subbiah et al[8] described a series of 15 patients. Ten patients developed neurologic manifestations. These findings included fatal hemorrhagic or ischemic strokes in 5, disabling polyradiculoneuropathy in 1, and nonspecific neurologic symptoms without objective findings in 4. Other manifestations include strokes, headaches, epilepsy, or nonspecific neurologic symptoms (eg, memory loss, altered sensation).
  • Patients experience abdominal pain. Gastrointestinal bleeding can result in vomiting blood or passing blood with bowel movements. Patients with Degos disease may have enterocutaneous fistulae.
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Causes

The cause of Degos disease is unknown. Suggested causes include a virus, an immune defect, or a clotting defect.

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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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  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].

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  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].

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  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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