Autoimmune bullous diseases represent a heterogenous group of disorders caused by autoantibodies against adhesion molecules; the location of the target protein determines the level of cleft formation. The spectrum of ocular lesions in autoimmune bullous diseases can range from mild symptoms to severe involvement with sight impairment and even, in some cases, blindness. In pemphigus vulgaris, the prevalence of ocular involvement has been reported to be between 7% and 26%. The most common clinical sign of ocular pemphigus vulgaris is bilateral conjunctivitis with hyperemia. Ocular involvement also occurs in 41% to 70% of patients with paraneoplastic pemphigus. The main ocular manifestations are bilateral cicatrizing conjunctivitis with symblepharon formation, and shortening of the fornices. In mucous membrane pemphigoid, ocular involvement is seen in 61% to 70% of patients; the most frequent ocular finding is cicatricial conjunctivitis. Patients with autoimmune bullous diseases having common ocular involvement should be assessed by an ophthalmologist to avoid serious complications. Diagnostic procedures and treatment require multidisciplinary care based on the close cooperation between dermatologists and ophthalmologists.
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http://dx.doi.org/10.1016/j.clindermatol.2023.08.007 | DOI Listing |
Ophthalmol Ther
January 2025
Corneoplastic Unit and Eye Bank, Queen Victoria Hospital NHS Foundation Trust, East Grinstead, UK.
Introduction: This study compared the clinical outcomes of allogenic cultured limbal epithelial transplantation (ACLET) and cultivated oral mucosal epithelial transplantation (COMET) in the management of limbal stem cell deficiency (LSCD).
Methods: Forty-one COMET procedures in 40 eyes and 69 ACLET procedures in 54 eyes were performed in the Corneoplastic Unit of Queen Victoria Hospital, East Grinstead. Data were examined for demographics, indications, ocular surface stability, absence of epithelial defect, ocular surface inflammation, visual outcomes, and intra- and postoperative complications.
Ophthalmol Ther
January 2025
Dr. Rolf M. Schwiete Center for Limbal Stem Cell and Congenital Aniridia Research, Saarland University, Homburg, Saar, Germany.
Introduction: Congenital aniridia is increasingly recognized as part of a complex syndrome with numerous ocular developmental anomalies and non-ocular systemic manifestations. This requires comprehensive care and treatment of affected patients. Our purpose was to analyze systemic diseases in patients with congenital aniridia within the Homburg Aniridia Registry.
View Article and Find Full Text PDFBMC Neurol
January 2025
Faculty of Medicine, Department of Neurology, Al-Quds University, Jerusalem, Palestine.
Background: Vanishing white matter disease (VWMD) is a rare autosomal recessive leukoencephalopathy. It is typified by a gradual loss of white matter in the brain and spinal cord, which results in impairments in vision and hearing, cerebellar ataxia, muscular weakness, stiffness, seizures, and dysarthria cogitative decline. Many reports involve minors.
View Article and Find Full Text PDFBMJ Case Rep
January 2025
Department of Ophthalmology, Rochdale Infirmary, Rochdale, UK.
Sweet syndrome (SS), or acute febrile neutrophilic dermatosis, is a dermatologic, auto-inflammatory disorder of unclear origin, often accompanied by systemic inflammation affecting various tissues, including the eyes. Common ocular manifestations include conjunctivitis but can extend to other ocular tissues. Orbital apex syndrome (OAS) involves damage to several cranial nerves transversing the orbital apex, leading to ophthalmoplegia and vision loss.
View Article and Find Full Text PDFAm J Ophthalmol
January 2025
Department of Ophthalmology and Visual Sciences, Kellogg Eye Center, University of Michigan, Ann Arbor, MI. Electronic address:
Purpose: To summarize and categorize postulated mechanisms of immune checkpoint inhibitor (ICI)-mediated retinal and choroidal inflammation and discuss resulting implications for evaluation and management of these adverse reactions.
Design: Targeted literature review with interpretation and perspective Methods: We performed a review of selected literature describing immune-mediated retinal and choroidal adverse reactions associated with ICI therapy, synthesizing and categorizing the likely underlying pathogenic mechanisms. Based on these mechanistic categories, we provide perspective on a rational approach to the evaluation of patients with ICI-associated inflammatory disorders of the retina and choroid.
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