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.
D001485:Basement Membrane / Q000648:ultrastructure; D004817:Epidermis / Q000648:ultrastructure; D006801:Humans; D008854:Microscopy, Electron; D010641:Phenotype; D011565:Psoriasis / Q000235:genetics / Q000473:pathology*; D012867:Skin / Q000648:ultrastructure*; D019896:alpha 1-Antitrypsin Deficiency