NEJM Case 38-2006 355:24 Dec 14, 2006 p.2575
Synopsis: A 5 yo boy developed HA, increased abdominal girth and a rash. Exam showed pappilledema. MRI showed subdural hygromas.The hygromas were bilateral and highly proteinaceous. He also had projectile vomiting. The HA were early day variety, relieved with tylenol and ppt by exercise. The rash was centripetal maculopapular. There were crops of papules on the trunk, arms and legs, sparing palms and soles,and face. Lesions are yellow or gray and become umbilicated with a central necrosis with a porcelain appearance. Lesions not seen can occur in the conjunctiva, sclera, choroids, retina and optic nerve. Gait is slightly ataxic.
Degos' disease was initially considered a cutaneous disease with a distinctive rash. Onset is from the first few months of life to the seventh decade. 70 % have a lethal, multisystem disease. COD is usually microvascular infarcts of the intestines.
Path-- noninflammatory vascular occlusive process mainly affecting arterioles. Specific cause of the disease is unknown; some think it is a marker of different diseases, including autoimmune diseases.
Pearls-- ascitic fluid taken was exudative, with a low SAAG (serum ascites albumen gradient 0.1). Negative cytology, AFB, lipase suggested a vasculitic cause. Repeat brain MRI showed hemorrhages at gray white junction and gyral edema with cystic encephalomalacia in the splenium. Additional lab showed d-dimer in 4000s, fibrinogen 600-1200, DIC. Treatment first with asa/dipyridamole, infliximab, later bevicizumab (monoclonal AB v. VEGF)
Synopsis: A 5 yo boy developed HA, increased abdominal girth and a rash. Exam showed pappilledema. MRI showed subdural hygromas.The hygromas were bilateral and highly proteinaceous. He also had projectile vomiting. The HA were early day variety, relieved with tylenol and ppt by exercise. The rash was centripetal maculopapular. There were crops of papules on the trunk, arms and legs, sparing palms and soles,and face. Lesions are yellow or gray and become umbilicated with a central necrosis with a porcelain appearance. Lesions not seen can occur in the conjunctiva, sclera, choroids, retina and optic nerve. Gait is slightly ataxic.
Degos' disease was initially considered a cutaneous disease with a distinctive rash. Onset is from the first few months of life to the seventh decade. 70 % have a lethal, multisystem disease. COD is usually microvascular infarcts of the intestines.
Path-- noninflammatory vascular occlusive process mainly affecting arterioles. Specific cause of the disease is unknown; some think it is a marker of different diseases, including autoimmune diseases.
Pearls-- ascitic fluid taken was exudative, with a low SAAG (serum ascites albumen gradient 0.1). Negative cytology, AFB, lipase suggested a vasculitic cause. Repeat brain MRI showed hemorrhages at gray white junction and gyral edema with cystic encephalomalacia in the splenium. Additional lab showed d-dimer in 4000s, fibrinogen 600-1200, DIC. Treatment first with asa/dipyridamole, infliximab, later bevicizumab (monoclonal AB v. VEGF)
