Detachment of keratinocytes at the basement membrane zone caused by inhibitory effect of the antibodies from sera of mucous membrane pemphigoid patients

2019 ◽  
Vol 46 (11) ◽  
pp. 1046-1048 ◽  
Author(s):  
Hiroshi Koga ◽  
Kwesi Teye ◽  
Norito Ishii ◽  
Takekuni Nakama
Author(s):  
Emily Davies

This chapter focuses on immunobullous diseases. The immunobullous disorders are a group of diseases in which pathogenic autoantibodies bind to target antigens either in desmosomes (intra-epidermal intracellular adhesion junctions) or in part of the basement membrane zone, resulting in loss of adhesion, and blister formation. This chapter will focus on pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, linear IgA disease, chronic bullous disease of childhood, and dermatitis herpetiformis; it will also mention mucous membrane pemphigoid, pemphigoid gestationis, and epidermolysis bullosa acquisita.


1985 ◽  
Vol 84 (2) ◽  
pp. 105-107 ◽  
Author(s):  
John R. Meyer ◽  
Cesar A. Migliorati ◽  
Troy E. Daniels ◽  
John S. Greenspan

2021 ◽  
Vol 97 (4) ◽  
pp. 193-198
Author(s):  
Nóra Belső ◽  
◽  
Lilla Mihályi ◽  
Zsuzsanna Zita Orosz ◽  
Zsuzsanna Bata-Csörgő

Mucous membrane pemphigoid (MMP) is an autoimmune subepithelial blistering disease. Oral and ocular mucosae are the most frequently afected areas, but the nasopharynx, esophagus, larynx and anogenital region can also be involved. It is characterized by linear deposition of IgG, IgA, or C3 along the basement membrane zone. In low-risk cases topical, intralesional corticosteroids are administered, together with anti-infammatory, immunomodulatory (dapsone) drugs or antibiotics (doxycycline). In severe, high-risk cases we apply systemic corticosteroids and immunosuppresive agents, or biological therapies (rituximab and anti-tumour necrosis factor drugs), or combination treatment of rituximab and intravenous immunoglobulins (IVIG)


Author(s):  
Narjes Akbari ◽  
Ghazaleh Mozafari ◽  
Hamid Abbaszadeh

Mucous membrane pemphigoid (MMP) is a rare inflammatory, autoimmune, and subepithelial vesiculobullous disease in which tissue-bound autoantibodies are produced against one or more components of the basement membrane. Oral lesions of the pemphigoid begin in the form of vesicles or bullae that often involve throughout the mouth but may be confined to specific areas, especially the gingiva, in a pattern known as desquamative gingivitis. The positive Nikolsky's sign is characteristic of pemphigus vulgaris, in which a blister can appear on the normal-appearing skin if exerting lateral pressure, and is very rare in the mucosa and other vesiculobullous diseases. Here we report a case of mucous membrane pemphigoid that developed as desquamated gingivitis in a 46-year-old woman with positive Nikolsky's sign in the gingival mucosa. In the histopathologic view, a subepithelial cleft was observed. The results of direct and indirect immunofluorescence tests and related therapeutic interventions are also presented. Positive Nikolsky's sign can be observed in the mucosa as well as in the mucous membrane pemphigoid in addition to pemphigus vulgaris, and vesiculobullous lesions should be diagnosed based on the sum of clinical, histopathological, and immunofluorescence findings.


Proceedings ◽  
2019 ◽  
Vol 35 (1) ◽  
pp. 57
Author(s):  
Gabusi ◽  
Loi ◽  
Gissi ◽  
Spinelli ◽  
Bernardi ◽  
...  

Mucous membrane pemphigoid (MMP) is a rare, predominantly mucosal subepithelialblistering disorder triggered by autoantibody reactivity to several basement membrane antigensincluding BP180, BP230, laminin 332, and type VII collagen [...]


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