Rituximab-Based Management of Autoimmune Blistering Diseases: A Case Series Highlighting Ocular and Systemic Presentations

利妥昔单抗治疗自身免疫性大疱性疾病:以眼部和全身表现为重点的病例系列

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Abstract

Mucous membrane pemphigoid (MMP), bullous pemphigoid (BP), and pemphigus vulgaris (PV) are rare autoimmune blistering disorders of the skin and mucous membranes that often result in delayed diagnosis and substantial morbidity. MMP is characterized by progressive conjunctival scarring, while BP typically presents with tense cutaneous blisters and PV with intraepidermal acantholysis driven by desmoglein autoantibodies. We present two women, aged 68 and 89 years, with ocular MMP, and one woman with PV, aged 48 years, highlighting the importance of early ophthalmic evaluation and prompt immunosuppressive therapy. Histopathology and immunofluorescence confirmed disease-specific patterns, with MMP demonstrating conjunctival fibrosis and subepithelial blistering, and PV showing vesiculobullous eruptions and antibody-mediated activity. All three patients responded favorably to rituximab, achieving steroid-sparing remission and stable disease control, underscoring its role as a key therapeutic option, particularly in vision-threatening ocular MMP. These findings emphasize the need for timely biopsy, comprehensive antibody testing, and early intervention, while supporting further research to standardize rituximab regimens and optimize long-term outcomes.

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