Electron microscopic features in psoriatic patients with alpha 1-antitrypsin deficiency

We studied the electron microscopic features of 7 psoriatic patients with alpha 1-antitrypsin deficiency and 14 psoriatic controls. We found a statistically significant difference in frequency of basal keratinocyte herniations (BKH) as well as of BKH with abnormal configuration (broad-based BKH, herniating through wide gaps in the basal lamina; and multipolypoid BKH) in the alpha 1-antitrypsin deficient group. The differences were more marked in the MZ phenotype than in the MS/SS phenotypes. These findings may reflect the changes resulting from defective inhibition of proteolytic enzyme activity in the psoriatic patients with alpha 1-antitrypsin deficiency, and support the concept that proteolytic enzyme release and activity may play a role in BKH formation.