Mucous membrane pemphigoid
Biju Vasudevan, Rajesh Verma in Dermatological Emergencies, 2019
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].
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).
A search for trachoma in Timor-Leste: no evidence to justify undertaking population-based prevalence surveys
Published in Ophthalmic Epidemiology, 2018
Marcelino Correia, David Brunner, Manoj Sharma, Valerio Andrade, Julia Magno, Andreas Müller, Bernardete M. Pereira, Gabriele Thumann, Nitin Verma, Mathieu Bangert, Andréas J. Kreis, Anthony W. Solomon
In the 2016 RAAB, all 13 districts of Timor-Leste were included, and 67 villages were selected with probability proportional to population size.39 Trained teams examined a total of 3253 participants aged ≥ 50 years.38 Four individuals, in Baucau, Cova Lima, and Liquiçá districts, were reported to have trachoma, including one female subject reported to have bilateral trachomatous corneal opacity.38 (During subsequent outreach work, this individual was identified, re-examined and found to have ankyloblepharon, without evidence of trichiasis or entropion.)
Related Knowledge Centers
- Amblyopia
- Blepharophimosis
- Chemical Burn
- Conjunctiva
- Eyelid
- Symblepharon
- Trisomy 18
- Prenatal Development
- Hay–Wells Syndrome
- Eye Injury