Immunopathologic investigations were conducted on the sera and oral mucosal tissue specimens of 23 patients with cicatricial pemphigoid. A linear, continuous basement membrane zone pattern was noted in 83% of oral mucosal biopsy specimens studied. This pattern is indistinguishable from the pattern noted in immunofluorescence studies of bullous pemphigoid, herpes gestationis, and some cases of desquamative gingivitis. Complement studies provided data supportive of classical pathway activation in cicatricial pemphigoid tissue. Deposition of IgA with Factor B, properdin, and C3 raised the possibility of alternative pathway activation, a question requiring further study. Circulating antibasement membrane zone antibodies were noted in the sera of two patients with cicatricial pemphigoid.
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http://dx.doi.org/10.1111/1523-1747.ep12485162 | DOI Listing |
Ophthalmol Ther
January 2025
Division of Rheumatology, University of Colorado School of Medicine, Aurora, CO, USA.
Introduction: Combined uveitis-rheumatology clinics (combined clinics) are a relatively recent clinical care model. Here we report the demographics, ocular and systemic disease characteristics, and medications utilized in patients seen in a combined clinic at a tertiary care hospital in the USA.
Methods: Medical records were reviewed of patients seen at the Combined Clinic at the University of Colorado Hospital between January 1, 2016 and November 1, 2023.
J Cutan Med Surg
December 2024
Division of Dermatology, Department of Medicine, McGill University and the McGill University Health Centre, Montréal, QC, Canada.
Cureus
November 2024
Dermatology, Texas Tech University Health Sciences Center, Lubbock, USA.
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.
View Article and Find Full Text PDFFront 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.
View Article and Find Full Text PDFOcul Immunol Inflamm
December 2024
University of Texas Southwestern Medical School, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
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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