Antiepiligrin cicatricial pemphigoid with autoantibodies to the beta subunit of laminin 5 and associated with severe laryngeal involvement necessitating tracheostomy.
We report the case of a 67-year-old Korean woman with antiepiligrin cicatricial pemphigoid. The patient's serum immunoprecipitated polypeptides that comigrated with those identified in serum from a representative patient with antiepiligrin cicatricial pemphigoid, and was reactive with the laminin beta3-subunit on immunoblotting. She presented not only with cutaneous, oral and ocular, but also with laryngeal and esophageal involvement. Because the supraglottic stenosis was severe, she had to undergo tracheostomy to maintain airway patency.
Seo
S H
SH
Department of Dermatology, Korea University College of Medicine, Seoul, South Korea. kudm@shinbiro.com
Kye
Y C
YC
Kim
S N
SN
Kim
S C
SC
D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001323:Autoantibodies / Q000097:blood*; D015815:Cell Adhesion Molecules / Q000276:immunology*; D003520:Cyclophosphamide / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007818:Laryngeal Diseases / Q000150:complications / Q000473:pathology* / Q000601:surgery; D010390:Pemphigoid, Benign Mucous Membrane / Q000150:complications / Q000188:drug therapy / Q000276:immunology*; D011239:Prednisolone / Q000627:therapeutic use; D014139:Tracheostomy; D000097794:Kalinin
D000893:Anti-Inflammatory Agents; D001323:Autoantibodies; D015815:Cell Adhesion Molecules; D003520:Cyclophosphamide; D007166:Immunosuppressive Agents; D011239:Prednisolone; D000097794:Kalinin