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Mucous membrane pemphigoid
Published in Biju Vasudevan, Rajesh Verma, Dermatological Emergencies, 2019
Dipankar De, Sheetanshu Kumar, Sanjeev Handa
Patients with conjunctival involvement initially present with unilateral disease and mild, nonspecific symptoms, such as burning, dryness, and foreign-body sensation (Figure 11.1) [27]. Later the disease may progress to involve both eyes in a period of about 2 years [28]. Gradually, fibrosis, conjunctival scarring, forniceal shortening, and symblepharon occur, later progressing to ankyloblepharon. Up to 20% of MMP patients have disease limited to conjunctiva, called ocular pemphigoid [27].
Topical Erythropoietin for Treatment of Scleral Necrosis
Published in Ocular Immunology and Inflammation, 2022
Sepehr Feizi, Mohammadhasan Alemzadeh-Ansari, Alireza Baradaran- Rafii, Hamed Esfandiari, Ahmad Kheirkhah
Five eyes in this series developed scleral ischemia after thermal or chemical burns. The main objectives for treatment of acute thermal and chemical burns are reduction of limbal stem cell damage, acceleration of conjunctival epithelialization and vascularization, and prevention of debilitating cicatricial sequelae. Timely management of the ischemic areas can prevent long-term cicatricial complications, including symblepharon and ankyloblepharon, as well as scleral perforation and ulcer. Our results indicate that topical erythropoietin could effectively promote epithelialization and vascularization of the ischemic areas caused by thermal and chemical burns, obviating the need for conjunctival graft and tenonplasty in already inflamed eyes. One of our cases who improved on topical erythropoietin developed extensive symblepharon formation. We believe that the symblepharon formation may partly be attributable to the delay in starting treatment with topical erythropoietin.
Incidence and Clinical Characteristics of Ocular Involvement in Mucous Membrane Pemphigoid
Published in Ocular Immunology and Inflammation, 2019
Gloria H. Hong, Irfan R. Khan, Amde Selassie Shifera, Chinwenwa Okeagu, Jennifer E. Thorne
Clinical data were collected on each patient, including demographic information, past ocular history, treatment, and clinical findings. Complete ophthalmic examination findings included best-corrected visual acuity (BCVA) or pinhole visual acuity, intraocular pressure (IOP), lid examination for entropion, ectropion, and trichiasis, grading of conjunctival cicatrization, and slit lamp assessment of the lid margin, conjunctiva, and cornea. In addition to examination of the mouth, nose and skin were examined, looking for the presence of extraocular MMP. Each patient was evaluated by a dermatologist, otorhinolaryngologist, and gastroenterologist for systemic manifestations of MMP. Conjunctival cicatrization was graded using the four-step Foster scale.5 Stage I is defined as the presence of chronic conjunctivitis with subepithelial fibrosis. Stage II consists of inferior fornix foreshortening. Stage III is characterized by the appearance of a symblepharon. Stage IV is end-stage disease with ankyloblepharon and extreme ocular surface keratinization. All data were recorded on a standardized data entry form for subsequent statistical analyses.
A rare form of ankyloblepharon filiforme adnatum associated with the Hay–Wells syndrome and a c.1709T>C mutation on the TP63 gene
Published in Ophthalmic Genetics, 2018
Michal Koubek, Kristýna Strakošová, Juraj Timkovič, Dagmar Grečmalová, Aneta Orlíková, Hana Burčková, Hana Wiedermannová, Petr Mašek
Hay–Wells (AEC) syndrome is an exceedingly rare disorder characterized by a wide spectrum of symptoms associated with the skin and skin adnexa to various degrees as well as with teeth, skin glands and extremities. Ankyloblepharon is a common symptom along with medium to severe skin erosions, abnormal hair, and lip and/or palate cleft. The syndrome is caused by mutations in TP63 gene on the 3q27 chromosome. Mostly, these mutations are “de novo”, however they can also be a hereditary autosomal dominant disease. The TP63 gene is encoding p63 protein, crucial for the development of extremities and ectoderm-derived structures such as hair, nails, skin glands, oral mucosa, etc., and its disruption can lead to the above mentioned disorders. (5,6).