RT Book, Section
A1 Yancey, Kim B.
A2 Goldsmith, Lowell A.
A2 Katz, Stephen I.
A2 Gilchrest, Barbara A.
A2 Paller, Amy S.
A2 Leffell, David J.
A2 Wolff, Klaus
SR Print(0)
ID 56038096
T1 Chapter 57. Cicatricial Pemphigoid
T2 Fitzpatrick's Dermatology in General Medicine, 8e
YR 2012
FD 2012
PB The McGraw-Hill Companies
PP New York, NY
SN 978-0-07-166904-7
LK accessmedicine.mhmedical.com/content.aspx?aid=56038096
RD 2023/06/07
AB |PrintCicatricial  Pemphigoid  at  a  GlanceA  chronic  autoimmune  subepithelial  blistering  disease  characterized  by  erosive  lesions  of  mucous  membranes  and  skin  that  result  in  scarring.Lesions  commonly  involve  the  oral  and  ocular  mucosae;  other  sites  that  may  be  involved  include  the  nasopharyngeal,  laryngeal,  esophageal,  genital,  and  rectal  mucosae.A  rare  disorder,  occurring  in  one  person  per  million  annually;  females  are  affected  1.5–2.0  times  as  often  as  males.A  progressive  disorder  that  may  result  in  serious  complications  (e.g.,  blindness,  loss  of  the  airway,  esophageal  stricture  formation).Immunopathologic  studies  of  perilesional  mucosa  and  skin  demonstrate  in  situ  deposits  of  immunoreactants  in  epithelial  basement  membranes;  circulating  antibasement  membrane  autoantibodies  are  detected  in  sera  of  some  but  not  all  patients.A  variety  of  different  autoantigens  are  recognized  by  autoantibodies  from  patients,  suggesting  that  cicatricial  pemphigoid  is  not  a  single  nosologic  entity  but  rather  a  disease  phenotype.