A patient with Wegener's granulomatosis with initial clinical presentations of Henoch-Schönlein purpura.

The initial presentation of a patient with Wegener's granulomatosis was indistinguishable from that of Henoch-Schönlein purpura. The patient presented with skin purpura and pulmonary hemorrhage followed by purpura in the colon. The diagnosis of this patient at that time was Henoch-Schönlein purpura. With time, massive lesions in the sinus and those with cavities in the lung became apparent, and a specimen obtained from the sinus massive lesion was disclosed to be granulomatous inflammation. Retrospectively, the proteinase 3 antineutrophil cytoplasmic antibody turned out to be strongly positive in her stored serum from the time of the initial presentation.