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. 2021 Jul 1;157(7):780-787.
doi: 10.1001/jamadermatol.2021.1036.

Assessment of Diagnostic Strategy for Mucous Membrane Pemphigoid

Hanan Rashid  1 , Joost M Meijer  1 , Gilles F H Diercks  2 , Nini E Sieben  1 , Maria C Bolling  1 , Hendri H Pas  1 , Barbara Horvath  1
Affiliations

Assessment of Diagnostic Strategy for Mucous Membrane Pemphigoid

Hanan Rashid et al. JAMA Dermatol. .

Abstract

Importance: An accurate diagnosis of mucous membrane pemphigoid (MMP) is essential to reduce diagnostic and therapeutic delay.

Objective: To assess the diagnostic accuracy of direct immunofluorescence microscopy on mucosal biopsy specimens and immunoserology in a large cohort of patients with suspected MMP.

Design, setting, and participants: This retrospective cohort study was carried out in a single tertiary care center for blistering diseases between January 2002 and March 2019. Eligible participants were patients with suspected MMP and paired data on at least a mucosal biopsy specimen for direct immunofluorescence microscopy (DIF) and indirect immunofluorescence microscopy (IIF) on a human salt-split skin substrate (SSS). In addition, an optional DIF test on a skin biopsy specimen and one or more performed routine immunoserologic tests were analyzed. Data analysis was conducted from April 2019, to June 2020.

Main outcomes and measures: Diagnostic accuracy of DIF, IIF SSS, and immunoblot for BP180 and BP230.

Results: Of the 787 participants, 121 (15.4%) received the diagnosis of MMP (50 men [41.3%], 71 women [58.7%]; mean [SD] age at diagnosis, 60.1 [17.7] years). Sixty-seven of the patients with MMP (55.4%) had monosite involvement, of which oral site was the most frequently affected (51 [42.1%]). No significant difference was found between the sensitivity of DIF on a perilesional buccal biopsy and a normal buccal biopsy (89.3% vs 76.7%). Three patients with solitary ocular involvement showed a positive DIF of only the oral mucosa. In 6 patients with a negative mucosal DIF, a skin biopsy confirmed diagnosis of MMP. Overall, IIF SSS was less sensitive (44.6%), but highly specific (98.9%). The sensitivity of immunoblot (66.1%) was higher compared to SSS, but with lower specificity (91.3%).

Conclusions and relevance: This comparative diagnostic accuracy study of a cohort of 787 patients found a high sensitivity of a mucosal DIF biopsy for diagnosis of MMP, and lower sensitivity of serologic analysis. A biopsy can be taken from either perilesional or normal buccal mucosa. An additional DIF biopsy of another mucosal site or of affected or unaffected skin may increase the diagnostic yield and is recommended in patients with negative DIF results and high clinical suspicion.

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Conflict of interest statement

Conflict of Interest Disclosures: Dr Horváth reports fees from Janssen-Cilag (advisory boards, educational grants, consultations, investigator initiative studies), AbbVie (advisory boards, educational grants, consultations, investigator initiative studies), Novartis Pharma (advisory boards, consultations, investigator initiative studies), UCB Pharma (advisory boards, consultations), Leo Pharma (Consultations), Solenne B.V. (investigator initiative studies), Celgene (consultations, investigator initiative studies), Akari therapeutics (consultations, investigator initiative studies), Philips (consultation), Roche (consultation), Regeneron (consultation) and Sanofi (consultation), which fees were paid to the institution. No other conflicts were reported.

Figures

Figure 1.
Figure 1.. Study Flow Diagram
Based on the index tests direct immunofluorescence (DIF) microscopy and indirect immunofluorescence on human salt-split skin (IIF SSS) substrate, eligible participants with other diagnoses were excluded. ELISA indicates enzyme-linked immunosorbent assays, ME indicates monkey esophagus.
Figure 2.
Figure 2.. Percentage of Positive DIF, IIF SSS, and immunoserologic Results in Patients With MMP
Skin biopsy for DIF was performed in 72 of 121 patients. DIF indicates direct immunofluorescence microscopy; ELISA, enzyme-linked immunosorbent assay; IIF SSS, indirect immunofluorescence microscopy on salt-split skin substrate; MMP, mucous membrane pemphigoid.
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