Degos Disease Treatment & Management

Updated: Feb 07, 2017
  • Author: Noah S Scheinfeld, JD, MD, FAAD; Chief Editor: Dirk M Elston, MD  more...
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Treatment

Medical Care

Until 2010, no medical therapy for Degos disease (DD) was noted 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. [45] This has changed with the reports that eculizumab can effectively treat systemic Degos disease. [1]

The initial enthusiasm for eculizumab as monotherapy may have faded. Burgin et al note that eculizumab (900 mg/d) failed as treatment for advanced systemic atrophic papulosis with GI involvement, with fatality as the outcome. [46] Shapiro et al note that only two patients initially given eculizumab as monotherapy are still alive; they instead use a combination of subcutaneous treprostinil and eculizumab. [47] Eculizumab, if given early enough, may help abate malignant atrophic papulosis, but as monotherapy it is not instrumental in controlling it.  With four patients treated, there have had no deaths. The first two are now six-year survivors. Each of them had been critically ill in ICU settings prior to therapy. This information is important because malignant atrophic papulosis without treatment or hope of treatment is lethal and patients should be directed towards those experienced with treatment as eculizumab alone may not work.

Other reports [48] have supported the use of eculizumab for systemic Degos disease but informal communication to the author noted that despite treatment with eculizumab, some patients with systemic Degos disease have died. One report noted 2 patients with Degos disease in whom eculizumab failed. [49] One had overlapping lupus and the other had pulmonary hypertension. The pulmonary hypertension patient was treated with treprostinil and cutaneous MAP lesions resolved but disabling digital pain manifested. In the patient with Degos disease and lupus, treprostinil temporally resulted in clearing of hematuria, CNS symptoms and MRI finding improvements.

A presentation by Magro et al [50] 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 support the idea that Degos disease 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.

An article published in 2013 found that exploratory treatment with eculizumab did not help the development or progression of Degos disease systemic manifestations, [51] with 5 of 7 patients’ disease initially improving but then regressing, with progression of disease in 1 immediately and the 4 others after a period of time.

A proposed new treatment for Degos disease is treprostinil, a medication for pulmonary hypertension. Shapiro et al (2013) report a series of 3 patients treated with subcutaneous treprostinil (dose not reported). [52] One patient had systemic lupus erythematosus (SLE), overlap scleroderma, extensive malignant atrophic papulosis lesions, and severe pulmonary hypertension without CNS or bowel involvement. She was placed on therapy with treprostinil for her pulmonary hypertension, but in the months subsequent to initiation of treatment, dramatic and complete resolution of cutaneous malignant atrophic papulosis–like lesions and disabling digital pain occurred. The second patient had pure malignant atrophic papulosis but progressive organ (CNS, bladder, neurological, and gross hematuria) dysfunction despite eculizumab treatment, but therapy with treprostinil was temporally associated with clearing of hematuria, resolution of CNS symptoms, and improvement in MRI findings.

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

Hohwy et al [40] 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.

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

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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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Long-Term Monitoring

Patients should be monitored for systemic manifestations of Degos disease and treated accordingly.

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