RESUMO
Congenital erythropoietic porphyria (CEP) is a rare autosomal recessive disorder in which the activity of uroporphyrinogen III synthase (UROS) is decreased. This results in the accumulation of photoreactive porphyrinogens, primarily in the skin and bone marrow. We describe a case of a patient with CEP who initially presented with scarring and shortening of the anterior and posterior lid lamella, which led to the development of lagophthalmos. Vascularized hyperkeratotic plaques in both corneas were also present. Despite treatment with topical ocular surface lubricants, corneal perforation with iris and uvea prolapse developed and evisceration of the right eye under local anesthesia was performed. The presented case suggests that despite topical therapy, ocular complications may exacerbate requiring surgical intervention, especially in the presence of lagophthalmos.
RESUMO
We present the first, unusual case of a lethal mediastinal hemorrhage caused by rupture of a saphenous vein aortic coronary bypass graft due to Aspergillus species necrotizing vasculitis in an immunocompetent patient 18 days after redo coronary artery bypass surgery. The patient had neither signs for mediastinitis nor for another source of Aspergillus infection.
