Abstract
A 24-year-old man was admitted to our department because of purpura on his extrimities, arthralgia and severe abdominal pain. Ultrasonography of the intestine revealed wide and severe inflammatory lesions on the jejunum. A histopathological examination of the skin lesions demonstrated leukocytoclastic vasculitis. He has no abnormality in the platelets or the intrinsic (PTT) and extrinsic (PT) pathways of coagulation, but the level of factor XIII activity in his serum was far below the normal range. The administration of omeprazole (20mg/day) and predonisolone (60mg/day) did not improve the symptoms. However, the abdominal pain promptly disappeared and the intestinal lesions improved after receiving factor XIII concentrate at a dose of 1200 U for 3 days. The administration of factor XIII concentrate has been mainly used for childhood cases of Schönlein-Henoch purpura with gastrointestinal symptoms, but our case suggests that it also appears to be effective in adult, cases especially in those with severe and steroid-resistant abdominal complications.
