Severe ulcerative panniculitis caused by alpha 1-antitrypsin deficiency: Remission induced and maintained with intravenous alpha 1-antitrypsin

Abstract

To the Editor: Panniculitis caused by homozygosity of ZZ alpha 1-antitrypsin (A1AT) deficiency is extremely rare despite the relatively common presence of ZZ phenotype affecting approximately 1 in 3500 people among whites in Western Europe. This phenotype deficiency can be associated with emphysema (greatly enhanced by smoking), hepatic cirrhosis, hepatitis, and angioedema.1 We present a case of a boy who developed extremely tender and debilitating recalcitrant panniculitis secondary to PiZZ phenotype A1AT deficiency requiring regular intravenous infusions of A1AT enzymes (Prolastin; Talecris Biotherapeutics, Research Triangle Park, NC) to maintain remission.