BACKGROUND: Degos disease is a type of vasculopathy characterized by progressive occlusion of small-sized blood vessels primarily involving the skin, gastrointestinal system, and central nervous system as well as various other systems. Owing to the rarity of the condition, the diagnosis is often a challenge; consequently, management is even more difficult owing to the paucity of experience and literature for the effective treatment of this entity. CASE PRESENTATION: We report a case of a 50-year-old male patient with classic skin lesions and rapidly progressive fatal clinical course involving multiple organs associated with dermatomyositis-like features. Therapeutic challenge in such a case of secondary Degos disease with multiorgan dysfunction is discussed. CONCLUSION: Secondary Degos disease with underlying multisystem dysfunction is a rapidly fatal disease. The patient must be thoroughly investigated to watch for systemic involvement and aggressively treated preferably with antiplatelet drugs and immunosuppresants so as to moderate the severity of the disease. Corticosteroids are best avoided to prevent gastrointestinal perforation.
BACKGROUND:Degos disease is a type of vasculopathy characterized by progressive occlusion of small-sized blood vessels primarily involving the skin, gastrointestinal system, and central nervous system as well as various other systems. Owing to the rarity of the condition, the diagnosis is often a challenge; consequently, management is even more difficult owing to the paucity of experience and literature for the effective treatment of this entity. CASE PRESENTATION: We report a case of a 50-year-old male patient with classic skin lesions and rapidly progressive fatal clinical course involving multiple organs associated with dermatomyositis-like features. Therapeutic challenge in such a case of secondary Degos disease with multiorgan dysfunction is discussed. CONCLUSION: Secondary Degos disease with underlying multisystem dysfunction is a rapidly fatal disease. The patient must be thoroughly investigated to watch for systemic involvement and aggressively treated preferably with antiplatelet drugs and immunosuppresants so as to moderate the severity of the disease. Corticosteroids are best avoided to prevent gastrointestinal perforation.
Authors: William Day; Christos Gabriel; Robert E Kelly; Cynthia M Magro; Judith V Williams; Alice Werner; Laura Gifford; Sameer P Lapsia; Cassyanne L Aguiar Journal: Rheumatol Int Date: 2020-01-03 Impact factor: 2.631