Granulomatosis sarcoidea necrosante con compromiso sistémico

Abstract

A 70-year-old mapuche female presented with a 1 year history of weight loss, bilateral red painful eyes and corneal ulcer that evolved into perforation and uveal herniation with complete visual loss. Reddish and painful nodules appeared on the distal aspect of both lower extremities, that ulcerated after months. A thoracic CT scan showed multiple pulmonary nodules. Infectious diseases were ruled out. An open lung biopsy and a skin biopsy were performed and both showed non-caseating necrotizing granulomas and granulomatous arteritis. Steroids and cyclophosphamide were started with complete regression of skin and pulmonary lesions within a month. Corneal transplantation was done. After 6 months follow-up, the patient is on low-dose of steroids, free of disease with partial vision recovery