Abstract
We report a 59-year-old Japanese male who developed recurrent urticaria and angioedema associated with respiratory manifestations. The patient had been regularly administered prednisolone (PSL) since he suffered from idiopathic interstitial pneumonia at 48 years of age. He also had membranoglomerulonephritis at 56 years of age. Dermatologically, he developed sausage-like fingers, sclerosis of the toes, a loss of toe nails and purpuric erythema on his palms and soles for 5 years and indurated erythema with alopecia of the scalp for 6 months. He thereafter developed an urticaria-like rash all over his body together with a high fever and dyspnea. The laboratory findings exhibited anemia, liver dysfunction, an accelerated erythrocyte sedimentation rate, hyperglobulinemia, a high level of anti-C1q immune complex and low level of complements. However, antinuclear antibody, anti-DNA antibody, and other specific antibodies for collagen diseases were all negative. A biopsy specimen from erythema of the left thigh showed a edematous dermis with features of leukocytoclastic vasculitis. The unaffected skin lacked the deposition of immunoglobulins or complements. The symptoms improved after an increasing dosage of PSL. Similar episodes recurred twice over the following 2 years, and pulmonary hemorrhaging occurred during the second event, and these symptoms improved after the administration of more than 10 grams of PSL. The serum complement levels thereafter returned to normal. Our case does not fulfil the criteria for a diagnosis of collagen disease and, as a result, this case is considered to most closely match the criteria for hypocomplementemic urticarial vasculitis syndrome.
