Structured Diagnostic Approach and Risk Assessment in Mucous Membrane Pemphigoid with Oesophageal Involvement

Benoit, Sandrine; Scheurlen, Michael; Goebeler, Matthias; Stoevesandt, Johanna

doi:10.2340/00015555-2938

Cited by 14 publications

(12 citation statements)

References 19 publications

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“…For the diagnosis of MMP, multiple examinations, including histological and immunological confirmation are required in most cases 1 . Tissue separation at the basement membrane zone (BMZ), which suggests subepithelial blisters in H&E staining is one of the most critical findings in the histological examination for MMP 11 . Immunological examinations are required to specify autoantibodies, particularly, tissue‐bound autoantibodies can be detected by direct immunofluorescence (DIF).…”

Section: Discussionmentioning

confidence: 99%

A case of anti‐laminin 332 mucous membrane pemphigoid manifesting as desquamative gingivitis

Maeda

Fujiwara

Tomihara

et al. 2020

Oral Science International

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Anti-laminin 332 mucous membrane pemphigoid (MMP) is characterized by circulating anti-laminin 332 autoantibodies, with a high risk of concurrent malignant neoplasms. We report a case of anti-laminin 332 MMP manifesting as a desquamative gingivitis. A 45-year-old woman experienced painful erythema and occasional bullous lesions on the gingiva despite good oral hygiene and plaque control. Intraoral examination revealed gingival inflammation with desquamation. Biopsy showed subepithelial cleft formation suggesting a subepidermal blister. Direct immunofluorescence revealed the deposition of IgG and IgA in the epithelium. Accordingly, we diagnosed anti-laminin 332 MMP and treated with systemic corticosteroids. The oral lesion resolved after 3 months.

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Section: Discussionmentioning

confidence: 99%

A case of anti‐laminin 332 mucous membrane pemphigoid manifesting as desquamative gingivitis

Maeda

Fujiwara

Tomihara

et al. 2020

Oral Science International

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“…In the acute attack of pemphigus vulgaris, oesophageal involvement was confirmed in 12/26 (46.2%) Turkish patients investigated 59 . In a German study of 30 patients with MMP, 9 out of 12 under examination was found to have oesophageal lesions 60 . A Brazil study of 12 patients with EBA demonstrated oesophageal involvement in 4 of 10 patients undergoing endoscopy 61 .…”

Section: Gi Involvement In Autoimmune Bullous Dermatosesmentioning

confidence: 95%

“…59 In a German study of 30 patients with MMP, 9 out of 12 under examination was found to have oesophageal lesions. 60 A Brazil study of 12 patients with EBA demonstrated oesophageal involvement in 4 of 10 patients undergoing endoscopy. 61 Pemphigus vulgaris and bullous pemphigoid are rare causes of oesophagitis dissecans superficialis (sloughing oesophagitis), which may lead to acute GI bleeding.…”

Section: Gi Involvement In Autoimmune Bullous Dermatosesmentioning

confidence: 99%

Gastrointestinal involvement of primary skin diseases

Hsieh

Wei

et al. 2020

Acad Dermatol Venereol

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Less is known about gastrointestinal (GI) involvement of primary skin diseases due to the difference in embryology, histology, microbiology and physiology between integument and alimentary tract. Oesophagus, following the oropharyngeal mucosa, is the most common GI segment affected by primary skin diseases, especially by eosinophilic oesophagitis, lichen planus and autoimmune bullous dermatoses like pemphigus vulgaris, mucosal membrane pemphigoid and epidermolysis bullosa acquisita. Eosinophilic oesophagitis is an emerging chronic atopic disease with oesophageal dysfunction as the typical presentation, and oesophageal narrowing, rings and stricture as late complications. Oesophageal lichen planus mainly involves the proximal to mid-oesophagus in elderly aged women with long-term oral mucosal lesions. In acute attack of pemphigus vulgaris, oesophageal involvement is not uncommon but often neglected and may cause sloughing oesophagitis (oesophagitis dissecans superficialis) with acute GI bleeding in rare cases. GI manifestation of hereditary bradykininergic angio-oedema with colicky acute abdomen mostly affects small intestine, usually in the absence of pruritus or urticaria, and is more severe and long-lasting than the acquired histaminergic form. Strong evidence supports association between inflammatory bowel disease, especially Crohn disease, and hidradenitis suppurativa/acne inversa. Patients with vitiligo need surveillance of autoimmune liver disease, autoimmune atrophic gastritis or coeliac disease when corresponding symptoms become suspect. Melanoma is the most common primary tumour metastatic to the GI tract, with small intestine predominantly targeted. Gastrointestinal involvement is not uncommon in disseminated mycosis fungoides. Extramammary Paget's disease is an intraepidermal adenocarcinoma of controversial origin, and a high association between the anogenital occurrence and colorectal adenocarcinoma has been reported. As GI tract is the largest organ system with multidimensional functions, dermatologists in daily practice should be aware of the gastrointestinal morbidities related to primary skin diseases for an early diagnosis and treatment.

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“…In addition, such examination does not always reveal subepithelial blisters, because of tissue destruction (27). Immunologically, the deposition of IgG autoantibodies and C3, or sometimes of IgA autoantibodies, at the BMZ can be detected by direct immunofluorescence (DIF) of lesional or perilesional samples (1, 28). To identify circulating autoantibodies to the BMZ, indirect immunofluorescence (IIF) with normal human skin as the substrate is usually performed, but autoantibodies are detected in only 17–53% of MMP cases (5, 17, 18, 29).…”

Section: Issues In Diagnosing Mmpmentioning

confidence: 99%

The Diagnosis and Blistering Mechanisms of Mucous Membrane Pemphigoid

Kamaguchi

Iwata

2019

Front. Immunol.

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Mucous membrane pemphigoid (MMP) is a mucous membrane-dominated autoimmune subepithelial blistering disease that is caused by autoantibodies against various autoantigens in basement membrane zone (BMZ) proteins, including collagen XVII (COL17). Clinicians face diagnostic problems in detecting circulating antibodies and targeted antigens in MMP. The diagnostic difficulties are mainly attributed to the low titers of MMP autoantibodies in sera and to heterogeneous autoantigens. Additionally, no unanimous diagnostic criteria have been drawn for MMP, which can result in delayed diagnoses or misdiagnoses. This review aims to integrate and present currently available data to clarify diagnostic strategies and to present diagnostic criteria for MMP. The ultimate blistering mechanism in MMP has not been elucidated, and such mechanism is especially obscure in COL17-type MMP. In bullous pemphigoid (BP), which is the most common autoimmune subepidermal blistering disease, some patients show oral lesion as well as predominant skin lesions. However, there is no fundamental explanation for the onset of oral lesions in BP. This article summarizes innovative research perspectives on the pathogenesis of oral lesions in pemphigoid. Finally, we propose a potential pathogenesis for COL17-type MMP.

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Structured Diagnostic Approach and Risk Assessment in Mucous Membrane Pemphigoid with Oesophageal Involvement

Cited by 14 publications

References 19 publications

A case of anti‐laminin 332 mucous membrane pemphigoid manifesting as desquamative gingivitis

A case of anti‐laminin 332 mucous membrane pemphigoid manifesting as desquamative gingivitis

Gastrointestinal involvement of primary skin diseases

The Diagnosis and Blistering Mechanisms of Mucous Membrane Pemphigoid

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