Mucous membrane pemphigoid (MMP) and its therapeutic strategies

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)

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Tumor Necrosis Factor-α in Ocular Mucous Membrane Pemphigoid and Its Effect on Conjunctival Fibroblasts

Investigative Opthalmology & Visual Science ◽

10.1167/iovs.08-3345 ◽

2009 ◽

Vol 50 (11) ◽

pp. 5310 ◽

Cited By ~ 20

Author(s):

Valerie P. J. Saw ◽

Robin J. C. Dart ◽

Grazyna Galatowicz ◽

Julie T. Daniels ◽

John K. G. Dart ◽

...

Keyword(s):

Tumor Necrosis Factor ◽

Mucous Membrane ◽

Tumor Necrosis ◽

Tumor Necrosis Factor Α ◽

Mucous Membrane Pemphigoid ◽

Factor Α ◽

Necrosis Factor

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Intravenous immunoglobulins for rituximab-resistant mucous membrane pemphigoid

Journal of the European Academy of Dermatology and Venereology ◽

10.1111/jdv.14873 ◽

2018 ◽

Vol 32 (8) ◽

pp. e321-e324 ◽

Cited By ~ 1

Author(s):

M. Witte ◽

D. Zillikens ◽

I. Shimanovich

Keyword(s):

Mucous Membrane ◽

Intravenous Immunoglobulins ◽

Mucous Membrane Pemphigoid

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Profile of autoantibody to basement membrane zone proteins in patients with mucous membrane pemphigoid: long-term follow up and influence of therapy

Clinical Immunology ◽

10.1016/j.clim.2004.04.010 ◽

2004 ◽

Vol 112 (3) ◽

pp. 268-272 ◽

Cited By ~ 22

Author(s):

Shih Wei Yeh ◽

Adnan Q Usman ◽

A.Razzaque Ahmed

Keyword(s):

Basement Membrane ◽

Mucous Membrane ◽

Basement Membrane Zone ◽

Mucous Membrane Pemphigoid ◽

Long Term Follow Up

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Unilateral Oral Mucous Membrane Pemphigoid: Refractory Atypical Presentation Successfully Treated with Intravenous Immunoglobulins

Case Reports in Dermatological Medicine ◽

10.1155/2015/930859 ◽

2015 ◽

Vol 2015 ◽

pp. 1-3 ◽

Cited By ~ 1

Author(s):

André Laureano ◽

Jorge Cardoso

Keyword(s):

Mucous Membrane ◽

Intravenous Immunoglobulins ◽

Mucous Membrane Pemphigoid ◽

Direct Immunofluorescence ◽

Oral Mucous Membrane ◽

Mucosal Involvement ◽

Oral Corticosteroids ◽

Local Trauma ◽

Palate Mucosa ◽

The Right

A 57-year-old male presented with a 6-month history of blisters and painful erosions on the right buccal mucosa. No skin or other mucosal involvement was seen. The findings of histopathological and direct immunofluorescence examinations were sufficient for the diagnosis of oral mucous membrane pemphigoid in the context of adequate clinical correlation. No response was seen after topical therapies and oral corticosteroids or dapsone. Intravenous immunoglobulin was started and repeated every three weeks. Complete remission was achieved after three cycles and no recurrence was seen after two years of follow-up. The authors report a rare unilateral presentation of oral mucous membrane pemphigoid on the right buccal and hard palate mucosa, without additional involvement during a period of five years. Local trauma or autoimmune factors are possible etiologic factors for this rare disorder, here with unique presentation.

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Mucous membrane pemphigoid: nasal and laryngeal manifestations

The Journal of Laryngology & Otology ◽

10.1258/002221503322542917 ◽

2003 ◽

Vol 117 (11) ◽

pp. 885-888 ◽

Cited By ~ 13

Author(s):

O. J. H. Whiteside ◽

P. Martinez Devesa ◽

I. Ali ◽

J. W. R. Capper

Keyword(s):

Mucous Membrane ◽

Scar Tissue ◽

Mucous Membrane Pemphigoid ◽

Mucosal Surfaces ◽

Laryngeal Mucosa ◽

Diagnostic Role ◽

Blistering Disease ◽

Low Threshold ◽

Laser Excision ◽

Prompt Action

Mucous membrane pemphigoid (MMP) is a sub-epithelial blistering disease that primarily involves mucosal surfaces but may also involve the skin. Clinically, it appears as vesiculobullous lesions of the oral cavity and eyes, but other tissues such as the nasopharyngeal and laryngeal mucosa can also be affected. Ultimately, scarring and airway stenosis may occur. The condition should be managed by a multidisciplinary team led by a dermatologist. Immunosuppressive therapy forms the mainstay of treatment, with surgery having both a diagnostic role and a use in the treatment of complications such as airway obstruction. There must be a low threshold of suspicion for laryngeal involvement in this group of patients, so that prompt action can be taken. Once laryngeal stenosis has occurred repeated endoscopic laser excision of scar tissue can be used to maintain an adequate airway. Adjuvant use of mitomycin-C can be used beneficially in the treatment of laryngeal complications of MMP.

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Systemic corticosteroid in the treatment of a 9-year-old boy with Oral Mucous Membrane Pemphigoid: A case report

Revista Odonto Ciência ◽

10.15448/1980-6523.2016.4.22911 ◽

2017 ◽

Vol 31 (4) ◽

pp. 186

Author(s):

Brunno Santos de Freitas Silva ◽

Jorge Elias Kaluf Tomeh ◽

Claudeir Felipe De Oliveira Siqueira ◽

Simone Sousa Silva Sant'Ana ◽

Fernanda Paula Yamamoto-Silva

Keyword(s):

Mucous Membrane ◽

Systemic Corticosteroid ◽

Mucous Membrane Pemphigoid ◽

Low Doses ◽

Oral Mucous Membrane ◽

Systemic Corticosteroids ◽

Attached Gingiva ◽

Burning Mouth Sensation ◽

Systemic Corticosteroid Therapy

AIM: This report is present a rare case of oral Mucous Membrane Pemphigoid (MMP) in a 9-yearold- boy successfully treated with low doses of systemic corticosteroid.CASE DESCRIPTION: A 9-year-old boy was referred to our service with the complaint of painful gingiva and generalized burning mouth sensation over 1 year. Intraoral examination revealed desquamative lesions affecting the entire attached gingiva of both the maxilla and the mandible. After the incisional biopsy the MMP diagnosis was confirmed, and the patient was initially treated with dexamethasone 0,1 mg/Ml mouth rinse twice daily for 24 weeks. Due the difficulty in eating and the presence of persistent large oral lesions, systemic prednisone 20 mg (1 mg/kg) was prescribed for 4 weeks with a 5 mg gradual reduction per week over 3 weeks. After initiating the systemic corticosteroid therapy, the patient showed total regression of the lesions, and no indication of recurrence has been observed in the past 6 months.CONCLUSION: There is no cure for MMP; however, treatment can provide complete and longlasting remission. Based on this premise, it is plausible to consider not only topical but also low doses of systemic corticosteroids in resistant cases of oral MMP, even in children. These measures could improve the quality of life of these patients by reducing pain and, consequently, improving the child’s eating behavior.

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