Degos Disease Medication

Updated: Dec 10, 2019
  • Author: Meagan O Harris, MD; Chief Editor: Dirk M Elston, MD  more...
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Medication

Medication Summary

Medical treatment of Degos disease is currently not well defined. Various agents have been tried, including antiplatelet agents, anticoagulants, immunosuppressive medications, fibrinolytic drugs, anti-inflammatory or vasodilator agents, and chloroquine, with varying degrees of success. [24] Several reports have described disease activity worsening during treatment with immunosuppressive agents. [56]

More recently, there has been hope for eculizumab, a monoclonal antibody targeting and inhibiting the C5 component of the complement cascade, as well as subcutaneous treprostinil, a vasodilatory agent [24] ; however, dosing and treatments guidelines have not been well established given the limited number of cases.

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Anticoagulant

Class Summary

Eculizumab has been found to be a life saving drug for those with systemic Degos disease.

Eculizumab (Soliris)

This is an off-label use for Degos disease. Eculizumab blocks complement-mediated destruction of paroxysmal nocturnal hemoglobinuria red blood cells. It inhibits the C5 component of the complement system, thereby preventing the final stages of complement activation.

The drug is FDA approved to reduce hemolysis with paroxysmal nocturnal hemoglobinuria and for atypical hemolytic uremic syndrome to inhibit complement-mediated thrombotic microangiopathy.

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Vasodilator

Class Summary

Prostacyclin is a strong vasodilator of all vascular beds and a potent endogenous inhibitor of platelet aggregation. Platelet effects result from activation of intracellular adenylate cyclase and from increased cyclic adenosine monophosphate (cAMP) concentrations within platelets. Prostacyclin may decrease thrombogenesis and platelet clumping in the lungs by inhibiting platelet aggregation.

Treprostinil (Tyvaso, Remodulin)

This is an off-label use for Degos disease. Treprostinil is a stable prostacyclin analogue. It elicits direct vasodilation of pulmonary and systemic arterial vessels and inhibits platelet aggregation. Vasodilation reduces right and left ventricular afterload and increases cardiac output and stroke volume.

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