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. 2021 Jul 26;35(10):1926–1948. doi: 10.1111/jdv.17395

Figure 1.

Figure 1

Diagnostic algorithm and work‐up, and diagnostic criteria for mucous membrane pemphigoid. 1Alternatively or in addition, direct immunoelectron microcopy can be performed. 2A positive DIF from any site is diagnostic for MMP, providing the clinical phenotype at the site that has not been biopsied is consistent with MMP. 3If ocular MMP is suspected, take biopsies from the least inflamed bulbar conjunctiva of both eyes together with another site (buccal mucosa or skin). Also take an additional lesional biopsy for routine histopathology to exclude both ocular surface neoplasia and sarcoid (which may present in the conjunctiva). 4Patients with predominant or exclusive IgA deposition could also be classified as having linear IgA disease. 5Patients with reactivity with type VII collagen could also be classified as having Epidermolysis Bullosa Acquisita. 6On human/primate salt‐split skin. 7Commercially available (for IgG antibodies). 8Only available in specialized diagnostic centers. 9Associated with a malignancy in 25–30% of patients; a tumor search is indicated. 10A diagnosis of immunopathology unconfirmed ocular monosite MMP can be made by exclusion of the more than 25 other causes of cicatrising conjunctivitis (CC). MMP is the most common cause of CC in most developed countries. Causes of CC, except for sarcoid & surface neoplasia: (i) have a history consistent with another cause of conjunctival disease; (ii) are positive on routine histopathology for neoplasia or sarcoid; or (iii) are DIF+ for another immuno‐bullous disease. If, after initiating appropriate therapy for immunopathology negative ocular monosite MMP, the disease course or response to therapy is not as expected, then this algorithm (both for DIF in ocular cases and serology) should be repeated and alternative diagnoses considered (e.g., severe ocular rosacea which can be difficult to differentiate from ocular MMP. DIF, direct immunofluorescence microscopy; ELISA, enzyme‐linked immuno sorbent assay; IIF, indirect immunofluorescence microscopy; MMP, mucous membrane pemphigoid.