ABSTRACT
PURPOSE: To present a rare and novel association of Ocular Cicatricial Pemphigoid, Sjögren's Syndrome, and Hashimoto's Thyroiditis as a Multiple Autoimmune Syndrome. CASE REPORT: A 43-year-old Colombian female, presented with corneal ulcers, associated with trichiasis. At the ophthalmological examination forniceal shortening OU and symblepharon OD was found. Conjunctival biopsy was performed, evidencing linear deposition of IgG and IgA antibodies along the basement membrane of the conjunctiva, confirming Ocular Cicatricial Pemphigoid diagnosis. After 12 years, the patient presented constitutional symptoms, xerostomia, and worsening of xerophthalmia. Laboratory tests showed positive Anti-TG, Anti-TPO, Anti-Ro, and Anti-La antibodies, and salivary gland biopsy was consistent with Sjögren's Syndrome. Due to these findings, Hashimoto's Thyroiditis and Sjögren's Syndrome were diagnosed, defining a Multiple Autoimmune Syndrome. CONCLUSION: A novel association of Multiple Autoimmune Syndrome is presented in this case. Ophthalmologists and other specialists involved in the evaluation and treatment of patients with autoimmune diseases, should be aware of this clinical presentation. A multidisciplinary approach in this condition is important for optimum treatment instauration and follow-up, in order to prevent complications.
Subject(s)
Pemphigoid, Benign Mucous Membrane , Sjogren's Syndrome , Thyroiditis , Adult , Biopsy , Conjunctiva/pathology , Female , Humans , Pemphigoid, Benign Mucous Membrane/complications , Pemphigoid, Benign Mucous Membrane/diagnosis , Pemphigoid, Benign Mucous Membrane/drug therapy , Sjogren's Syndrome/complications , Sjogren's Syndrome/diagnosis , Thyroiditis/complicationsABSTRACT
AIM: To provide an epidemiological characterization of ocular cicatricial pemphigoid (OCP) in Colombia. METHODS: We conducted a cross-sectional study using SISPRO. We applied the specific code of the International Classification of Diseases for Ocular Pemphigoid, from 2009-2019 to estimate prevalence, incidence, and the demographic status of the disease in Colombia. RESULTS: The estimated average prevalence was 0.22 per 1 000 000 inhabitants, and the estimated average incidence was 0.24 per 1 000 000 inhabitants. With a female predominance of 62.5%, and a male/female ratio of 1:1.6. The group of patients diagnosed with the disease after the age of 80 presented the highest prevalence. The departments with the highest prevalence were Antioquia, Bogotá, and Santander. CONCLUSION: There are important differences between worldwide and Colombian prevalence and incidence data, which may be related to genetic and epigenetic factors, and the possible underdiagnosis of the disease. According to the results, OCP is an extremely rare disease in Colombia. Nevertheless, it is important to encourage awareness of the disease due to its devastating consequences.
