TY - JOUR
T1 - S2k Guideline for the diagnosis and treatment of mucous membrane pemphigoid
AU - Hofmann, Silke C.
AU - Günther, Claudia
AU - Böckle, Barbara C.
AU - Didona, Dario
AU - Ehrchen, Jan
AU - Gaskins, Matthew
AU - Geerling, Gerd
AU - Gläser, Regine
AU - Hadaschik, Eva
AU - Hampl, Monika
AU - Haßkamp, Pia
AU - Jackowski, Jochen
AU - Kiritsi, Dimitra
AU - Nast, Alexander
AU - Pleyer, Uwe
AU - Reichel, Christoph
AU - Roth, Mathias
AU - Schumann, Michael
AU - Sticherling, Michael
AU - Worm, Margitta
AU - Zillikens, Detlef
AU - Goebeler, Matthias
AU - Schmidt, Enno
N1 - Publisher Copyright:
© 2022 The Authors. Journal der Deutschen Dermatologischen Gesellschaft published by John Wiley & Sons Ltd on behalf of Deutsche Dermatologische Gesellschaft.
PY - 2022/11
Y1 - 2022/11
N2 - Mucous membrane pemphigoid (MMP) is a pemphigoid disease with predominant mucous membrane involvement. It mainly affects the mucous membranes of the mouth, eyes, nose and pharynx, but also the larynx, trachea, esophagus, genital and perianal regions. The manifestation of the disease covers a wide spectrum from gingival erythema and single oral lesions to severe tracheal strictures that obstruct breathing and conjunctival scarring with marked visual impairment and, not infrequently, blindness. In addition to a clinical picture of predominant mucosal involvement, diagnosis is based on direct immunofluorescence of a peri-lesional biopsy and serology. The main target antigen is BP180 (collagen XVII), and reactivity with laminin 332 is associated with malignancy in approximately 25 % of MMP patients. The treatment of MMP is challenging. On the one hand, due to the involvement of different mucous membranes, good interdisciplinary cooperation is required; on the other hand, due to the rarity of the disease, no randomized controlled clinical trials are available. The aim of this guideline is to present the clinical picture, including severity and scoring systems, and to give guidance for diagnosing and treating this complex disease. In MMP, interdisciplinary cooperation plays an essential role as well as the prompt diagnosis and initiation of adequate therapy in order to avoid irreversible damage to the mucous membranes with serious complications.
AB - Mucous membrane pemphigoid (MMP) is a pemphigoid disease with predominant mucous membrane involvement. It mainly affects the mucous membranes of the mouth, eyes, nose and pharynx, but also the larynx, trachea, esophagus, genital and perianal regions. The manifestation of the disease covers a wide spectrum from gingival erythema and single oral lesions to severe tracheal strictures that obstruct breathing and conjunctival scarring with marked visual impairment and, not infrequently, blindness. In addition to a clinical picture of predominant mucosal involvement, diagnosis is based on direct immunofluorescence of a peri-lesional biopsy and serology. The main target antigen is BP180 (collagen XVII), and reactivity with laminin 332 is associated with malignancy in approximately 25 % of MMP patients. The treatment of MMP is challenging. On the one hand, due to the involvement of different mucous membranes, good interdisciplinary cooperation is required; on the other hand, due to the rarity of the disease, no randomized controlled clinical trials are available. The aim of this guideline is to present the clinical picture, including severity and scoring systems, and to give guidance for diagnosing and treating this complex disease. In MMP, interdisciplinary cooperation plays an essential role as well as the prompt diagnosis and initiation of adequate therapy in order to avoid irreversible damage to the mucous membranes with serious complications.
UR - http://www.scopus.com/inward/record.url?scp=85142148649&partnerID=8YFLogxK
U2 - 10.1111/ddg.14905
DO - 10.1111/ddg.14905
M3 - Journal articles
C2 - 36354061
AN - SCOPUS:85142148649
SN - 1610-0379
VL - 20
SP - 1530
EP - 1550
JO - JDDG - Journal of the German Society of Dermatology
JF - JDDG - Journal of the German Society of Dermatology
IS - 11
ER -