Panniculitis caused by a 4-antitrypsin deficiency
O. Yu Olisova , O. V Grabovskaya , N. P Teplyuk , T. A Belousova , I. S Dzhavakhishvili , V. A Varshavsky
Russian Journal of Skin and Venereal Diseases ›› 2014, Vol. 17 ›› Issue (3) : 32 -35.
Panniculitis caused by a 4-antitrypsin deficiency
Presents data on panniculitis and its different forms with special emphasis on panniculitis caused by а 1-antitrypsin (а 1-AT) deficiency, difficulties in the diagnosis and treatment of this condition. The diagnosis ofа -AT deficiency is based on clinical laboratory studies (common analysis of blood, biochemical study, ultrasonic study, esophagogastroduodenoscopy, etc.), measurement of blood а -AT concentration, and molecular genetic studies - detection of PiZZ genotype (Glu342Lys mutation in homozygotic state). The results of authors ’ observations are presented: effective systemic glucocorticosteroid therapy of panniculitis caused by а -AT deficiency.
panniculitis / molecular genetic study / systemic glucocorticosteroids
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