elastin and Epidermolysis-Bullosa

Topics: Animals; Basement Membrane; Cattle; Collagen; Connective Tissue; Ehlers-Danlos Syndrome; Elastin; Epidermolysis Bullosa; Fibroblasts; Glycopeptides; Humans; Keloid; Lysine; Marfan Syndrome; Metabolism, Inborn Errors; Microbial Collagenase; Mixed Function Oxygenases; Progeria; Protein Biosynthesis; Pseudoxanthoma Elasticum; Wound Healing

Specimens from a patient with epidermolysis bullosa contained many elastic globes in the dermis. Ultrastructurally they were composed of (i) medium electron-dense amorphous substances, (ii) electron-dense round structures, and (iii) fine filaments. These various elements were seldom organized into typical normal elastic fiber and, therefore, it was difficult ultrastructurally to recognize them as such or components thereof. Immunohistochemically, elastic globes were strongly reactive with NKH-1, which stains elastic microfibrils, and antibody to serum amyloid P component (anti-SAP), which binds to elastic fiber microfibrils. However, elastic globes were negative with EKH-4 which recognizes 50 kd keratin of amyloid keratin and cytoid bodies. These findings suggested that elastic globes have a close immunologic profile to elastic fiber microfibrils, but not that of epidermal or epithelial keratin.

Topics: Adult; Antibodies, Monoclonal; Elastic Tissue; Elastin; Epidermolysis Bullosa; Female; Humans; Immunohistochemistry; Keratins; Microscopy, Electron

Topics: Amino Acids; Amyloidosis; Collagen; Elastic Tissue; Elastin; Epidermolysis Bullosa; Epidermolysis Bullosa Acquisita; Geriatrics; Hexosamines; Histocytochemistry; Humans; Pathology; Skin