- Indian Rheumatology association
P Damodaram DM
Consultant Rheumatologist, Subodaya Rheumatology Hospital, Tirupati
A young female presented with recurrent painful ulcers over the lower leg around the malleolus at the age of 18 years. Antinuclear antibodies, antiphospholipid antibodies, antineutrophilic cytoplasmic antibodies, and complement levels were negative. She has previously received cyclophosphamide, IVIG, mycophenolate mofetil, and cyclosporine for refractory cutaneous vasculitis. The skin biopsy showed fibrin deposition and fibrin thrombus in superficial small vessels.
Answer:
DEGOS DISEASE is a rare disease of unknown etiology categorized as a benign cutaneous to lethal multisystem vasculopathy. It is also reported to involve the gut, brain, kidneys, lungs, pleura, and liver and the prognosis depends on the systemic involvement. It usually begins with a long-term cutaneous disease. Bowel perforation is the commonest cause of death. Immunosuppression plays no role in these cases, and the use of steroids is known to increase the severity of the disease.
