2009, Number 6
<< Back Next >>
Rev Mex Oftalmol 2009; 83 (6)
Prevalencia de manifestaciones oculares de la epidermolisis bulosa en México
Stevenson M, Salas J, Páez JH, Rodríguez-García A
Language: Spanish
References: 13
Page: 333-339
PDF size: 181.67 Kb.
ABSTRACT
Purpose: To determine the most frequent ocular manifestations in patients with epidermolysis bullosa in Mexico, and compare them with other published studies.
Design: Observational cross-sectional.
Methods: 23 Mexican patients from Instituto DEBRA México A.C. with diagnosis of recessive dystrophic epidermolysis bullosa underwent a complete ophthalmologic examination. The results were compared with the ones reported in the literature, using squared-chi(X2).
Results: The study included 16 females (69.6%), and 7 males (30.4%) patients. With a mean age of 17.1 years (range 1-59 years). The 52.1% of the patients were found asymptomatic. The most frequent symptoms were: ocular irritation 30.4%, tearing 13.0% and palpebral bullae 8.6%. At the ophthalmic examination we found: blepharitis in 60.8%, meibomian gland dysfunction in 43.4%, symptomatic refractive errors in 39.1%, corneal scarring in 39.1%, and epithelial keratitis in 26.0%.
Conclusions: The Recessive dystrophic epidermolysis bullosa is a rare genodermatosis, with formation of spontaneous bullae and ulcers that produce scarring. Patients must have a multidisciplinary treatment, with special attention to the external ocular surface to prevent scarring that can impair vision.
REFERENCES
Matsumoto Y, Dogru M, Tsubota K. Ocular surface findings in Hallopeau-Siemens subtype of dystrophic epidermolysis Bulosa: report of a case and literature review. Cornea 2005; 24(4):474-479. Review. PubMed PMID: 15829808.
Herron GS. Epidermolysis Bulosa. Eye and Skin Disease. Mannis MJ, Macsai MS and Huntley AC. (Eds). Lippincott-Raven Publishers Philadelphia, PA. 1996. Chap. 35; pp. 285.
Featherstone C. Epidermolysis Bulosa: from fundamental molecular biology to clinical therapies. J Invest Dermatol 2007; 127:256-259.
Salas-Alanis JC. Las Epidermolisis bulosas. El proyecto DEBRA. Med Cutan Iber Lat Am 2007; 35:165-166
Fine JD, Bauer EA, McGuire J y cols. Epidermolysis Bulosa. Clinical, epidemiological and laboratory advances and the findings of the National Epidermolysis Bulosa Registry. Baltimore: John Hopkins University Press, 1999.
Fine JD, Eady RA, Bauer EA, Bauer JW, Bruckner-Tuderman L, Heagerty A y cols. The classification of inherited epidermolysis Bulosa (EB): Report of the Third International Consensus Meeting on Diagnosis and Classification of EB. J Am Acad Dermatol 2008; 58(6):931-950. Epub 2008 Apr 18.
Fine JD, Johnson LB, Weiner M, Stein A, Cash S, Deleoz J, Devries DT, Suchindran C. Eye involvement in inherited epidermolysis Bulosa: experience of the National Epidermolysis Bulosa Registry. Am J Ophthalmol. 2004; 138(2):254-262. PubMed PMID: 15289135.
Khan AO. Corneal ulcer in a young child with autosomal recessive epidermolysis Bulosa. J Pediatr Ophthalmol Strabismus 2006; 43(6):370-372. PubMed PMID: 17162976.
Tong L, Hodgkins PR, Denyer J, Brosnahan D, Harper J, Russell- Eggitt I, Taylor DS, Atherton D. The eye in epidermolysis Bulosa. Br J Ophthalmol 1999; 83(3):323-326. PubMed PMID: 10365041; PubMed Central PMCID: PMC1722980.
Van Bijsterveld O. Diagnostic tests in the sicca syndrome. Arch Ophthalmol 1969; 82:10-14.
Lin AN, Murphy F, Brodie SE, Carter DM. Review of ophthalmic findings in 204 patients with epidermolysis Bulosa. Am J Ophthalmol 1994; 118(3):384-390. Review. PubMed PMID: 8085596.
Gans LA. Eye lesions of epidermolysis Bulosa. Clinical features, management, and prognosis. Arch Dermatol 1988; 124(5):762-764. PubMed PMID: 3364998.
McDonnell PJ, Spalton DJ. The ocular signs and complications of epidermolysis Bulosa. J R Soc Med 1988; 81(10):576-578. PubMed PMID: 3184090; PubMed Central PMCID: PMC1291800.