Abstract
A 76-year-old female was admitted to our hospital with 3-days history of purpuric lesions preceded by sore throat. Numerous purpura with hemorrhagic blisters was noted on her lower limbs including the dorsum of feet. A skin biopsy of purpura on her lower leg was performed and showed leukocytoclastic vasculitis with perivascular infiltrates of neutrophils and lymphocytes in the superficial and middle dermis. Depositions of IgA and C3 on the vascular walls were detected by direct immunofluorescence. The laboratory findings revealed a marked decrease of factor XIII and proteinuria. Titers of ASO were high with detection of Streptococ.alpha-hemolytic on throat swab culture so that she was diagnosed with anaphylactoid purpura following streptococcal infection. By administration of antibiotics, her purpuric lesions were improved in a few days,but from the day 6 of illness, a number of purpura and proteinuria relapsed. At the same time, nasal and palpebral conjunctiva hemorrhage developed. The laboratory data indicated the presence of DIC based on the DIC criteria scoring up to 7 points. We treated her with steroid pulse therapy, thrombomojulin and supplementation of the factor XIII. The purpura and hemorrhage disappeared with oral prednisolone 40 mg/day followed by steroid pulse therapy, but the serum creatinine levels still elevated. Renal biopsy examination revealed that mesangium cell proliferation, IgA, C3 and fibrinogen deposition in the glomerulus, by which we diagnosed of purpura nephritis. We performed steroid pulse therapy, cyclophosphamide pulse therapy, plasmapheresis and LDL apheresis, which resulted in some recovery of renal function. We propose that the decrease in the serum levels of fator XIII may be a useful predictive factor for determining the severity of IgA vasculitis.
