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Summary: We describe a case of the heterozygous antitrypsin phenotype IS. The 20 years old female employee had been recurrently suffering from cutaneous vasculitis over a period of three years. Serum trypsin Inhibitor was found to be high (1394 ImU) during the acute stage of the disease. No other serological or physical abnormalities were detected. A relationship between certain Pi (the term Pi indicating protease Inhibitor = OL\ -antitrypsin) alleles and vasculitis is possible but not certain, as three out of four other patients with this disease were MM phenotypes, the other one being MS type.

A Case of Rare Heterozygous α 1 -Antitrypsin Phenotype: IS