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Mucous membrane pemphigoid (MMP) is a rare autoimmune subepithelial blistering disorder characterized by predominant involvement of the mucosae, a chronic course, and a tendency toward scarring of the affected areas.1 A relatively common sign of MMP is desquamative gingivitis. We describe our experience using rituximab to treat 3 patients with recalcitrant desquamative gingivitis as the sole manifestation of MMP. Diagnosis of MMP was based on clinical and immunopathological criteria.1,2 The MMP mucosal activity score (MAS), which was used for the evaluation of the response, was retrospectively calculated based on the clinical pictures and notes.2
Patient characteristics, immunopathological findings, and responses to therapy are summarized in the Table. The patients had been previously treated with a combination of doxycycline and nicotinamide; dapsone, up to 100 mg/d; sulfasalazine, 2 g/d; prednisolone, 0.5 mg/kg/d; and/or mycophenolate mofetil, 2 g/d. These regimens, in addition to topical corticosteroids, careful oral hygiene, and avoidance of trauma, failed to control disease activity, and so the patients were subsequently given rituximab (MabThera; Roche) in 2 infusions of 1000 mg each with an interval of 2 weeks between infusions.
Rituximab treatment was followed by a complete remission in patients 1 and 2 (MAS, 0 of 120) at the 6-month follow-up examination (Figure). The third patient showed partial remission: his MAS decreased from 10 of 120 to 4 of 120, while the dose of sulfasalazine was reduced from 2 g/d to 1 g/d. At the 24-month follow-up, 1 patient was in complete remission off treatment, while 2 patients were in complete remission on minimal adjuvant therapy (Table).
A, Before rituximab treatment, manifestations of erosive gingivitis as a sign of mucous membrane pemphigoid include gingival mucosal edema and erosions. B, At the 6-month follow-up examination, the gingival mucosal edema and erosions have completely regressed, leaving some gingival retraction and mucosal atrophy.
There are anecdotal cases and small case series describing a favorable effect of rituximab on MMP with life- and sight-threatening complications.3,4 A recent review3 found that 20 of 28 patients with MMP treated with rituximab had a complete response, while 3 had a partial response. Nevertheless, at least half of the patients needed a second cycle of rituximab because of either relapse or lack of response. Le Roux-Villet et al4 describe a series of 25 patients with refractory or severe MMP treated with rituximab who experienced significant benefit for ocular disease. A recent study of 49 patients with moderate to severe MMP provided evidence that addition of rituximab to conventional therapy was associated with more rapid and sustained disease control.5 However, data about isolated erosive gingivitis, which may represent the sole manifestation of MMP, are missing. Our experience with 3 patients indicates that a single cycle of rituximab is a beneficial second-line therapeutic option in patients with isolated chronic erosive gingivitis and does not cause severe complications while effectively treating a condition that would otherwise require long-term immunosuppressive therapies.
Corresponding Author: Stefanie Häfliger, MD, Dermatologie Inselspital, Freiburgstrasse 14A, 3010 Bern, Bern, Switzerland (email@example.com, firstname.lastname@example.org).
Published Online: October 12, 2016. doi:10.1001/jamadermatol.2016.3434
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank the patients for granting permission to publish this information.
Haefliger S, Horn MP, Suter VGA, Bornstein MM, Borradori L. Rituximab for the Treatment of Isolated Refractory Desquamative Gingivitis Due to Mucous Membrane Pemphigoid. JAMA Dermatol. 2016;152(12):1396–1398. doi:10.1001/jamadermatol.2016.3434