Degos Disease Medication

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

For systemic Degos disease, the treatment of choice is eculizumab. The dosing of eculizumab is likely above the current FDA-approved dose for other conditions. Three cases of the successful use of eculizumab have been reported, and others likely exist. One aspect hindering use of eculizumab in Degos disease is the drug's high cost. Eculizumab is not a treatment for purely cutaneous Degos disease.

Some authorities have used antiplatelet medications, but their ultimate effect is unclear.



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.



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.