In-vivo-Konfokalmikroskopie in der Diagnose des okulären Schleimhautpemphigoids

Hecht, Eva-Maria; Pitz, Susanne; Renieri, Giulia

doi:10.1055/s-0035-1546137

Cited by 3 publications

(3 citation statements)

References 9 publications

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“…Circulating antibodies have been detected in a small percentage of patients on monkey oesophagus substrate, ranging from 2.6% to 8%. 28 , 29 , 36 Normal human skin had previously demonstrated higher values (17–35%). 25 , 31 , 37 In the salt‐split skin technique, normal human or primate skin is incubated in 1 mol/L NaCl until splitting occurs within the lamina lucida of the BMZ.…”

Section: Diagnosticsmentioning

confidence: 91%

“… 22 In case of MMP, positivity is defined by the detection of linear IgG or IgA along the BMZ of different tissue substrates. 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 Compared to bullous pemphigoid, IgG positivity by IIF in MMP occurs less frequently and usually with lower titres. 25 , 27 , 31 , 32 This may be due to the heterogeneity of the target antigens, or the lower amount of autoantibodies in MMP sera.…”

Section: Diagnosticsmentioning

confidence: 99%

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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

110

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: Diagnosticsmentioning

confidence: 91%

Section: Diagnosticsmentioning

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

110

109

View full text Add to dashboard Cite

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“…Reports regarding the sensitivity of DIF in MMP have been inconclusive, demonstrating a wide range from 41% to 100% for mucosal biopsies ( 10 , 18 – 21 ), and 44% to 100% for skin samples ( 10 , 18 , 20 , 21 ). Notably, in previous studies, circulating autoantibodies have been detected in only 2.6% to 8% of patients with MMP using monkey oesophagus substrate ( 22 – 24 ), and in 36% to 84% with 1 M sodium chloride-split normal human skin ( 15 , 23 , 25 – 29 ). Owing to the comparably low sensitivity of DIF and IIF ( 11 ), the definitive diagnosis of MMP has posed a core challenge.…”

Section: Discussionmentioning

confidence: 93%

Evaluation of the Diagnostic Value of Oesophageal Biopsies for Direct Immunofluorescence Microscopy in Mucous Membrane Pemphigoid

Yilmaz,

Dikmen,

Van Beek

et al. 2023

Acta Derm Venereol

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Mucous membrane pemphigoid is an autoimmune blistering disorder characterized by predominant involvement of surface-close epithelia and linear depositions of immunoreactants at the dermal-epithelial junction on direct immunofluorescence microscopy. A major diagnostic difficulty is the frequent need for multiple biopsies to facilitate the diagnosis. Although oesophageal involvement is a rare, but life-threatening manifestation, the relevance of oesophageal direct immunofluorescence sampling is unclear. This retrospective monocentric study evaluated 67 non-lesional biopsies from 11 patients with mucous membrane pemphigoid and clinical symptoms suggestive of oesophageal involvement, comprising 31 samples from the oesophagus and 36 samples from other anatomical sites. Five patients (45.5%) exhibited endoscopic findings compatible with oesophageal involvement of mucous membrane pemphigoid. No correlation was identified between the presence of oesophageal lesions and direct immunofluorescence positivity in lesions from the oesophagus (p = 1.0). Oral and cutaneous samples were significantly more frequently positive by direct immunofluorescence than were oesophageal biopsies (p < 0.0001 and p = 0.0195, respectively). Oesophageal samples yielded significantly less IgG reactivity than oral and cutaneous lesions (p < 0.0001 and p = 0.0126, respectively), and less IgA antibody response than oral lesions (p = 0.0036). In conclusion, oesophageal direct immunofluorescence samples were inferior to oral and cutaneous biopsies for the diagnosis of mucous membrane pemphigoid even when oesophageal lesions compatible with mucous membrane pemphigoid were present at the time of biopsy.

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A review of scoring systems for ocular involvement in chronic cutaneous bullous diseases

Lee

Tan

Radjenovic

et al. 2018

Orphanet J Rare Dis

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BackgroundEpidermolysis bullosa (EB) and autoimmune blistering diseases (AIBD) describe a group of rare chronic dermatoses characterized by cutaneous fragility and blistering. Although uncommon, significant ocular surface disease (OSD) may occur in both and require ophthalmological assessment. Disease scoring systems have a critical role in providing objective and accurate assessment of disease severity. The objectives of this report were, firstly, to document the prevalence and severity of ocular involvement in EB/AIBD. Secondly, to review and evaluate existing ocular and systemic scoring systems for EB/AIBD. Finally, to identify areas where further development of ocular specific tools in EB/AIBD could be pursued.MethodsA literature search was performed in October 2017 utilising Medline, Embase, and Scopus databases. The results were restricted by date of publication, between 01.01.1950 and 31.10.2017. The reference lists of these articles were then reviewed for additional relevant publications. Articles of all languages were included if an English translation was available. Articles were excluded if they were duplicates, had no reference to ocular involvement in EB/AIBD or described ocular involvement in other diseases.ResultsDescriptions of ocular involvement in EB/AIBD were identified in 88 peer-reviewed journal articles. Findings reported include but are not limited to: cicatrising conjunctivitis, meibomian gland dysfunction, dry eye disease, trichiasis, symblepharon, fornix fibrosis, keratopathy, ectropion/entropion, ankyloblepharon, corneal ulceration, visual impairment and blindness. Although scoring systems exist for assessment of OSD in mucous membrane pemphigoid, no such tools exist for the other AIBD subtypes or for EB. Several systemic scoring systems exist in the dermatological literature that are efficacious in grading overall EB/AIBD severity, but have limited inclusion of ocular features. To the best of our knowledge, there is no recognised or validated scoring systems which comprehensively stages or grades the spectrum of ocular manifestations in EB/AIBD.ConclusionsThere are a range of ocular complications documented in EB and AIBD. Development of a comprehensive ocular scoring system for EB/AIBD which incorporates the delineation between ‘activity’ and ‘damage’ would facilitate more objective patient assessment, improved longitudinal monitoring, comparison of intervention outcomes, and provide commonality for discussion of these patients due to the multidisciplinary nature of their care.

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In-vivo-Konfokalmikroskopie in der Diagnose des okulären Schleimhautpemphigoids

Abstract: In-vivo confocal microscopy may serve as a useful additional diagnostic method in the detection of MMP.

Cited by 3 publications

References 9 publications

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

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

Evaluation of the Diagnostic Value of Oesophageal Biopsies for Direct Immunofluorescence Microscopy in Mucous Membrane Pemphigoid

A review of scoring systems for ocular involvement in chronic cutaneous bullous diseases

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