Intravenous immunoglobulins for rituximab-resistant mucous membrane pemphigoid

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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Efficacy of intravenous immunoglobulins for the treatment of mucous membrane pemphigoid-like epidermolysis bullosa acquisita

European Journal of Dermatology ◽

10.1684/ejd.2017.3118 ◽

2017 ◽

Vol 27 (5) ◽

pp. 563-564 ◽

Cited By ~ 3

Author(s):

Takaya Komori ◽

Teruki Dainichi ◽

Nobuhiro Kusuba ◽

Atsushi Otsuka ◽

Takashi Hashimoto ◽

...

Keyword(s):

Mucous Membrane ◽

Epidermolysis Bullosa ◽

Intravenous Immunoglobulins ◽

Epidermolysis Bullosa Acquisita ◽

Mucous Membrane Pemphigoid

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Mucous membrane pemphigoid and COVID-19 treated with high-dose intravenous immunoglobulins: a case report

Journal of Dermatological Treatment ◽

10.1080/09546634.2020.1764472 ◽

2020 ◽

Vol 31 (5) ◽

pp. 446-447 ◽

Cited By ~ 7

Author(s):

Maryam Daneshpazhooh ◽

Tahereh Soori ◽

Ahdie Isazade ◽

Pedram Noormohammadpour

Keyword(s):

Case Report ◽

Mucous Membrane ◽

Intravenous Immunoglobulins ◽

Mucous Membrane Pemphigoid ◽

High Dose

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Mucous membrane pemphigoid (MMP) and its therapeutic strategies

Bőrgyógyászati és Venerológiai Szemle ◽

10.7188/bvsz.2021.97.4.3 ◽

2021 ◽

Vol 97 (4) ◽

pp. 193-198

Author(s):

Nóra Belső ◽

◽

Lilla Mihályi ◽

Zsuzsanna Zita Orosz ◽

Zsuzsanna Bata-Csörgő

Keyword(s):

Mucous Membrane ◽

Tumour Necrosis ◽

Intravenous Immunoglobulins ◽

Therapeutic Strategies ◽

Basement Membrane Zone ◽

Mucous Membrane Pemphigoid ◽

Systemic Corticosteroids ◽

Anogenital Region ◽

Blistering Disease ◽

Necrosis Factor

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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