ocular pemphigoid

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...

Alport syndrome (AS) is a hereditary progressive glomerular disease associated with sensorineural hearing loss and ocular abnormalities. It is attributed to the altered structure and the subsequent dysfunction of the glomerular basement membrane (GBM) due to the mutated type IV collagen a3/a4/a5 chains. It may emerge either as an X-linked disease, the most common, or as an autosomal disease, both recessive and dominant. A female patient, 26 years old, came in 2023 to the Department of Oral Medicine/ Pathology, Dental School, Aristotle University of Thessaloniki, complaining about pain and a burning sensation in her right cheek...

Mucous membrane pemphigoid refers to a heterogeneous group of autoimmune diseases with subepidermal blister formation that can affect all mucous membranes with varying frequencies. This is a rare disease without any geographic or sexual predisposition that is characterized by recurrent inflammation and progressive scarring. The specific diagnostics can be negative in up to 50% of cases. The diagnosis is predominantly made in patients aged 60-80 years. Ophthalmologists play an important role in the care of affected individuals as the conjunctiva is the second most frequent site of involvement...

PURPOSE: The aim of this study was to describe the presenting feature of vortex keratopathy in 3 patients with biopsy-proven ocular mucous membrane pemphigoid (OMMP). METHODS: The first patient was a 52-year-old woman with chronic redness and foreign body sensation for 3 years who presented with unilateral vortex keratopathy. Seven months later, in the same eye, she developed conjunctival signs suggestive of OMMP. The second patient was a 33-year-old woman with similar chronic symptoms that were exacerbated by pterygium surgery...

PURPOSE: To evaluate preferred diagnostic tools and treatment decision-making factors in cases suspicious of mucous membrane pemphigoid (MMP) amongst ophthalmologists and cornea specialists. METHODS: Web-based survey, consisting of 14 multiple choice questions, posted to the Cornea Society Listserv Keranet, the Canadian Ophthalmological Society Cornea Listserv, and the Bowman Club Listserv. RESULTS: One hundred and thirty-eight ophthalmologists participated in the survey...

This study evaluates the long-term outcomes of a cohort of 205 patients undergone modified osteo-odonto-keratoprosthesis, confirming the efficacy of this technique with anatomical and functional survival rates of 81.1% and 59.6% at 40 years respectively.

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Ocular mucous membrane pemphigoid is the manifestation of a systemic autoimmune disease. As autoantibodies circulating in the blood cannot be adequately treated by eye drops, systemic immunosuppressive treatment of this autoimmune disease naturally plays the most important role. Ophthalmic topical or even surgical procedures are only used as supportive measures or usually to control ocular complications that have developed. Patients with the typical clinical picture are treated causally with systemic immunosuppression causally, nurturing eye drops, as well as by minimally invasive surgery if complications arise, if possible in an inflammation-free state, in accordance with the guidelines if the diagnosis is positive but also if the biopsy and serology are repeatedly negative after exclusion of all differential diagnoses...

No abstract text is available yet for this article.

The diagnosis of ocular mucous membrane pemphigoid (MMP) remains a challenge as the timing and choice of diagnostic methods have a decisive influence on its quality. A systematic approach is required that includes a comprehensive medical history, critical evaluation of the clinical findings and targeted laboratory testing. The diagnosis is complicated by the fact that some patients present purely clinically the symptoms of MMP without fulfilling the required immunohistochemical and laboratory criteria...

Mucous membrane pemphigoid (MMP) is a rare disease that presents clinicians with a diagnostic and therapeutic challenge. The aim of this article is to present the German ocular pemphigoid register, which is a retrospective data collection and a collaborative network to improve the care of these patients. It was founded in 2020 and currently comprises 17 eye clinics/cooperation partners. An initial evaluation of the results shows a known epidemiological profile and an expected high proportion of patients with negative diagnostics (48...

Overall, conjunctival scarring is rare and often overlooked during routine examination. However, the differential diagnostic distinction of cicatricial mucous membrane pemphigoid from other causes is crucial for further treatment planning. Other entities that lead to conjunctival scarring, are ocular rosacea, ocular graft-versus-host disease, atopic keratoconjunctivitis, infections, trauma and further sometimes very rare diseases. Most of these differential diagnoses have in common that conjunctival scarring does not progress indefinitely if untreated, in contrast to mucous membrane pemphigoid...

Sebaceous carcinoma is a known mimicker of benign conditions, leading to frequent delays in diagnosis and proper treatment. We present two patients with chronic cicatrizing conjunctivitis initially diagnosed as ocular mucous membrane pemphigoid (MMP) and later found to have sebaceous carcinoma. Both patients presented with unilateral conjunctivitis that failed to improve with topical and systemic therapy, eventually developing fornix foreshortening and extensive symblepharon. Case 1 was diagnosed with ocular MMP based on clinical features alone, while Case 2 was diagnosed with biopsy-negative disease...

Mucosal pemphigoid is a rare chronic autoimmune disease that also affects the eyes in more than two thirds of all cases. Especially in the early phase of the ocular manifestation, the findings are subtle and the disease is often not recognized. The aim of this article is to provide the clinical aspects of ocular mucosal pemphigoid so that timely diagnostics can be initiated when this disease is suspected.

OBJECTIVES: To evaluate the demographic data, ocular and systemic findings, clinical management, and outcomes of patients with ocular cicatricial pemphigoid (OCP). MATERIALS AND METHODS: The medical records of 11 patients diagnosed as having OCP in the ophthalmology department of Ege University between 2008 and 2021 were evaluated retrospectively. RESULTS: The patients' mean follow-up time was 14±5.76 months. All eyes (100%) had conjunctival involvement and 18 (81...

OBJECTIVE: To investigate the long-term outcomes of fornix reconstruction and cicatricial entropion repair in patients with ocular mucous membrane pemphigoid (MMP) and secondary MMP. METHODS: Retrospective chart review of patients with MMP undergoing either fornix reconstruction (with amniotic membrane or buccal mucosal graft) or Wies cicatricial entropion repair between January 1, 2000, and September 1, 2020. Patients had a positive mucosal biopsy and (or) clinical features of MMP or secondary MMP...

Immunosuppression in aqueous-deficient dry eye disease (ADDE) is required not only to improve the symptoms and signs but also to prevent further progression of the disease and its sight-threatening sequelae. This immunomodulation can be achieved through topical and/or systemic medications, and the choice of one drug over the other is determined by the underlying systemic disease. These immunosuppressive agents require a minimum of 6-8 weeks to achieve their beneficial effect, and during this time, the patient is usually placed on topical corticosteroids...

Cataract surgery, which is the most widely performed ophthalmic procedure, is usually done in the elderly population, who are also prone to ocular surface disorders. Ocular surface diseases are multifactorial in nature and associated with symptoms and signs such as foreign body sensation, burning, fatigue, photophobia, red or watery eyes, or reduced visual acuity. These include a spectrum of conditions that may be immune or non-immune in nature. Cataract surgery in itself is known to alter the normal ocular surface milieu and cause tear film disturbances which can last up to 6 months post-operatively...

Bilateral corneal blindness with severe dry eye disease (DED), total limbal stem cell deficiency with underlying corneal stromal scarring and vascularization, combined with adnexal complications secondary to chronic cicatrizing conjunctivitis is a highly complex situation to treat. In such eyes, procedures such as penetrating keratoplasty alone or combined with limbal stem cell transplantation are doomed to fail. In these eyes, keratoprosthesis (Kpro) or an artificial cornea is the most viable option, eliminating corneal blindness even in eyes with autoimmune disorders such as Stevens-Johnson syndrome, ocular mucous membrane pemphigoid, Sjogren's syndrome, and nonautoimmune disorders such as chemical/thermal ocular burns, all of which are complex pathologies...

INTRODUCTION: We describe a series of patients whose auto-immune bullous skin disease (AIBD) of the dermal-epidermal junction (DEJ) was characterized by clinical, immunological and ultrastructural features intermediate between bullous pemphigoid (BP) and mucous membrane pemphigoid (MMP), and a recalcitrant course. PATIENTS AND METHODS: From the database of the French reference centre for AIBD, we screened all the patients who were referred for an AIBD of the DEJ with a mucosal involvement, who neither met the diagnostic criteria for the diagnosis of BP, nor were typical of MMP...