eMedicine Specialties > Dermatology > Diseases of the Vessels

Degos Disease: Follow-up

Author: Noah S Scheinfeld, MD, JD, FAAD, Assistant Clinical Professor, Department of Dermatology, Columbia University; Consulting Staff, Department of Dermatology, New York Medical College-Metropolitan Hospital; Private Practice
Contributor Information and Disclosures

Updated: Feb 14, 2008

Follow-up

Further Inpatient Care

  • Care is primarily supportive. Admission to the hospital can be necessary in cases of gastrointestinal bleeding or other systemic complications.

Further Outpatient Care

  • Patients should be monitored for systemic manifestations of DD and treated accordingly.

Inpatient & Outpatient Medications

  • The role of medications is unclear. Patients might need transfusions if intestinal bleeding is present and, antibiotics, if an abdominal perforation is present.

Transfer

  • Patients who manifest with systemic symptoms must be transferred to a center that can provide appropriate care.

Complications

  • Complications of DD include the following:
    • Gastrointestinal bleeding and perforation
    • Neurologic bleeding and collapse
    • Cardiac pathologic conditions
    • Pulmonary pathologic conditions
    • Ocular complications

Prognosis

  • Patients with only skin lesions may have a good prognosis.
  • Gastrointestinal involvement may occur in as many as 60% of patients, and death in such cases is likely.
  • About 15% of patients with DD have long-term survival, with disease often limited to the skin and with no manifestations of fatal bowel or CNS involvement.

Patient Education

  • Because systemic DD is fatal, patients and their families should be educated in this regard.

Miscellaneous

Medicolegal Pitfalls

  • Failure to diagnosis the condition is a pitfall. Although the treatment of systemic DD does not change its disease course or cure DD, treatment of gastrointestinal and neurologic complications prolongs the patient's life.

Special Concerns

  • DD can occur in infants. DD has been reported in a 7-month-old girl who showed spontaneous aggregation of platelets. A good clinical response in this patient was obtained by treatment with aspirin and dipyridamole.
  • In patients with benign cutaneous DD, immunosuppression (in 1 patient with cyclosporine) can cause DD to evolve into systemic fatal disease.
 


More on Degos Disease

Overview: Degos Disease
Differential Diagnoses & Workup: Degos Disease
Treatment & Medication: Degos Disease
Follow-up: Degos Disease
Multimedia: Degos Disease
References
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References

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Further Reading

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Keywords

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

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Contributor Information and Disclosures

Author

Noah S Scheinfeld, MD, JD, FAAD, Assistant Clinical Professor, Department of Dermatology, Columbia University; Consulting Staff, Department of Dermatology, New York Medical College-Metropolitan Hospital; Private Practice
Noah S Scheinfeld, MD, JD, FAAD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

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, Lipoplasty Society of North America, Southwest Pediatric Nephrology Study Group, Southwestern Oncology Group, Southwestern Surgical Congress, Special Operations Medical Association, State Medical Society of Wisconsin, Swedish Medical Association, Sydenham Society, Tennessee Medical Association, Tennessee Radiological Society, Texas Medical Association, Texas Pediatric Society, Texas Society of Plastic Surgeons, Undersea and Hyperbaric Medical Society, Uniformed Services Academy of Family Physicians, United States and Canadian Academy of Pathology, United States Pharmacopeial Convention, US Virgin Islands Medical Society, Utah Medical Association, Vermont State Medical Society, Vestibular Disorders Association, Virginia Society of Otolaryngology-Head and Neck Surgery, West Virginia State Medical Association, Western Occupational and Environmental Medical Association, Western Orthopaedic Association, Western Section American Urological Association, Western Surgical Association, Wilderness Medical Society, World Association of Societies of Pathology and Laboratory Medicine, World Medical Association, World Society for Stereotactic and Functional Neurosurgery, and Wyoming Medical Society
Disclosure: Nothing to disclose.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Director Dermatology Residency Training Program, Scott and White 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: 3M Pharmaceutical Grant/research funds Other; Graceway Pharmaceuticals Grant/research funds Other

Managing Editor

Jeffrey J Miller, MD, Associate Professor, Department of Dermatology, Penn State University, Milton S Hershey Medical Center
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 None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

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.

 
 
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