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We present the case of a 36-year-old paraplegic woman with a history of spinal cord injury who developed a generalized blistering rash, later diagnosed as bullous pemphigoid (BP). During her hospitalization, she was treated with prednisone and rituximab infusions, transitioning to maintenance therapy with topical steroids, doxycycline, and nicotinamide. A year later, she presented with concerns about a BP flare on her feet.

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Bullous pemphigoid and mucous membrane pemphigoid humoral responses differ in reactivity towards BP180 midportion and BP230.

Front Immunol

December 2024

Molecular and Cell Biology Laboratory, Istituto Dermopatico dell'Immacolata (IDI)-IRCCS, Rome, Italy.

Background: Bullous pemphigoid (BP) and mucous membrane pemphigoid (MMP) are rare autoimmune blistering disorders characterized by autoantibodies (autoAbs) targeting dermo-epidermal junction components such as BP180 and BP230. The differential diagnosis, based on both the time of appearance and the extension of cutaneous and/or mucosal lesions, is crucial to distinguish these diseases for improving therapy outcomes and delineating the correct prognosis; however, in some cases, it can be challenging. In addition, negative results obtained by commercially available enzyme-linked immunosorbent assays (ELISAs) with BP and MMP sera, especially from patients with ocular involvement, often delay diagnosis and treatment, leading to a greater risk of poor outcomes.

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Purpose: To develop a novel grading scale for assessing conjunctival inflammation in cicatrizing conjunctivitis associated with Pemphigoid.

Methods: We performed a retrospective analysis of digital slit-lamp images for patients with cicatricial pemphigoid in which best-quality conjunctival images for each eyelid at each photographic session was selected and anonymized. These images were subsequently prospectively assessed for inflammation severity on a scale of 0-4+ by a fellowship-trained ophthalmologist specializing in pemphigoid.

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Article Synopsis
  • The study introduces a surgical technique called oral mucous membrane tarsal patch grafting (MMTPG) for treating tarsal surface abnormalities beyond the eyelid margin.
  • It includes a retrospective analysis of 8 patients with various refractory tarsal conjunctival conditions, focusing on outcomes like corneal epitheliopathy, visual acuity, and patient symptoms.
  • Results showed no recurrences of epithelial defects, improved visual acuity in five cases, and reduced medication needs, indicating MMTPG's effectiveness in difficult-to-treat conditions.
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