Mucous Membrane Pemphigoid of the Vulva

Goldstein, Andrew T.; Anhalt, Grant J.; Klingman, Daisy; Burrows, Lara J.

doi:10.1097/01.aog.0000147837.85831.6c

Cited by 21 publications

(7 citation statements)

References 7 publications

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“…Two patients required systemic corticosteroids, sulphones, azathioprine and dapsone 143 . Topical therapy in adult MMP is often not sufficient to achieve remission of genital lesions 144,145 …”

Section: Managementmentioning

confidence: 99%

European Guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology – Part II

Schmidt

Rashid

Marzano

et al. 2021

Acad Dermatol Venereol

115

109

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This guideline has been initiated by the task force Autoimmune Blistering Diseases of the European Academy of Dermatology and Venereology, including physicians from all relevant disciplines and patient organizations. It is a S3 consensus‐based guideline that systematically reviewed the literature on mucous membrane pemphigoid (MMP) in the MEDLINE and EMBASE databases until June 2019, with no limitations on language. While the first part of this guideline addressed methodology, as well as epidemiology, terminology, aetiology, clinical presentation and outcome measures in MMP, the second part presents the diagnostics and management of MMP. MMP should be suspected in cases with predominant mucosal lesions. Direct immunofluorescence microscopy to detect tissue‐bound IgG, IgA and/or complement C3, combined with serological testing for circulating autoantibodies are recommended. In most patients, serum autoantibodies are present only in low levels and in variable proportions, depending on the clinical sites involved. Circulating autoantibodies are determined by indirect IF assays using tissue substrates, or ELISA using different recombinant forms of the target antigens or immunoblotting using different substrates. The major target antigen in MMP is type XVII collagen (BP180), although in 10–25% of patients laminin 332 is recognized. In 25–30% of MMP patients with anti‐laminin 332 reactivity, malignancies have been associated. As first‐line treatment of mild/moderate MMP, dapsone, methotrexate or tetracyclines and/or topical corticosteroids are recommended. For severe MMP, dapsone and oral or intravenous cyclophosphamide and/or oral corticosteroids are recommended as first‐line regimens. Additional recommendations are given, tailored to treatment of single‐site MMP such as oral, ocular, laryngeal, oesophageal and genital MMP, as well as the diagnosis of ocular MMP. Treatment recommendations are limited by the complete lack of high‐quality randomized controlled trials.

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Section: Managementmentioning

confidence: 99%

European Guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology – Part II

Schmidt

Rashid

Marzano

et al. 2021

Acad Dermatol Venereol

115

109

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“…Genital involvement in MMP can be misdiagnosed as lichen sclerosus et atrophicus, lichen planus, pemphigus or even sexual abuse. 80 , 82 , 85 , 86 , 87 , 88 , 89 , 90 Distinguishing it from localized vulvar pemphigoid may also pose a challenge. Genital involvement in MMP can be drug‐induced or paraneoplastic.…”

Section: Clinical Presentationmentioning

confidence: 99%

European guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology – Part I

Rashid

Lamberts

Borradori

et al. 2021

Acad Dermatol Venereol

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This guideline on mucous membrane pemphigoid (MMP) has been elaborated by the Task Force for Autoimmune Blistering Diseases of the European Academy of Dermatology and Venereology (EADV) with a contribution of physicians from all relevant disciplines and patient organizations. It is a S3 consensus-based guideline encompassing a systematic review of the literature until June 2019 in the MEDLINE and EMBASE databases. This first part covers methodology, the clinical definition of MMP, epidemiology, MMP subtypes, immunopathological characteristics, disease assessment and outcome scores. MMP describes a group of autoimmune skin and mucous membrane blistering diseases, characterized † Both authors contributed equally.

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“…Leonard et al postulated that the presence of tissue eosinophils in LS, particularly eosinophilic spongiosis, may be an indication of coexistent bullous pemphigoid. Clinical features of vulvar pemphigoid may resemble those of LS . Most recently, Lester and Swick found that eosinophils are particularly common in male genital lesions of LS and in LS associated with squamous cell carcinoma.…”

mentioning

confidence: 99%