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Mary Tien Labowsky, Christine Shieh, Jason Liss, Melissa Daluvoy, Russell Hall; A Retrospective Review of the Clinical Implications of Biopsy Results in Mucous Membrane Pemphigoid. Invest. Ophthalmol. Vis. Sci. 2016;57(12):499.
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© ARVO (1962-2015); The Authors (2016-present)
Mucous Membrane Pemphigoid (MMP) is diagnosed clinically or by biopsy demonstrating linear IgG, IgA or C3 at the dermal-epidermal junction on direct immunofluorescence (DIF); however, a negative biopsy result does not preclude a clinical diagnosis. We investigated whether patients with negative biopsy results differ from those with biopsy-proven disease in terms of areas involved and disease progression in a retrospective cohort study of patients seen at a tertiary multi-disciplinary Mucous Membrane Pemphigoid clinic.
We identified 53 patients diagnosed with ocular MMP that were followed for at least 1 year (between 1996 and 2015) with DIF biopsy results, excluding those with a history of Stevens-Johnson syndrome, Toxic Epidermal Necrolysis, and suspected pseudopemphigoid. Data collected included patient demographics, signs and symptoms, visual acuity, physical exam findings, and DIF biopsy results ("positive" linear IgG, IgA, or C3, or "negative", no deposits or linear fibrin alone). Foster’s Stages and MMP Disease Activity Index (MMPDAI) were assigned based on reported physical exam findings at initial presentation and at 18-months. Patients with insufficient documentation at 18-months were excluded from analysis.
Of 25 patients who presented with ocular disease alone, 14 (56%) had positive biopsy findings and 11 (44%) had negative findings. Of 28 patients with combined ocular, skin or mucosal disease, 26 (92.3%) had positive biopsies and 2 (7.1%) had negative biopsies. The proportion of patients with negative biopsies was significantly higher in those with ocular disease alone compared to those with other tissues involved (p=0.004). There was no significant difference between patients with positive and negative biopsies when comparing progression in Foster’s Stage, MMPDAI, or visual acuity from presentation to 18 months.
Compared to patients with both ocular and extra-ocular manifestations, patients with isolated ocular MMP had a higher proportion of negative biopsies. Over 18-months, patients with negative biopsies had a similar clinical course as those with positive biopsies in terms of progression in Foster’s stages, MMPDAI, and visual acuity. Our data suggests that negative biopsy results are more likely to be found in MMP patients with isolated ocular disease, but that these patients have similar disease course as patients with positive biopsy results.
This is an abstract that was submitted for the 2016 ARVO Annual Meeting, held in Seattle, Wash., May 1-5, 2016.
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