Agustin España1, Pilar Iranzo2, Josep Herrero-González3, José M Mascaro2, Ricardo Suárez4. 1. Department of Dermatology, Unit of Autoimmune Blistering Skin Diseases; University Clinic of Navarra, School of Medicine, Pamplona, Navarra, Spain. 2. Hospital Clinic of Barcelona, Spain. 3. Hospital del Mar of Barcelona, Spain. 4. Hospital Gregorio Marañon of Madrid, Spain.
Abstract
BACKGROUND AND OBJECTIVES: Ocular/periocular involvement in pemphigus vulgaris (OPV) has rarely been reported. The objective of the present study was to investigate the pattern of OPV and define the prognostic value of its manifestation. PATIENTS AND METHODS: From 1985 to 2014, a total of 167 patients with pemphigus vulgaris (PV) were treated at four tertiary Spanish hospitals. In this retrospective study, we included all patients with OPV. Clinical data and information on associated symptoms were obtained from patients' medical records. RESULTS: Only 24 (14.3 %) of all PV patients had ocular lesions. In most cases, -ocular involvement was preceded by PV lesions at various other sites (mean duration: 33.7 months). Ocular PV lesions occurred during flares of mucocutaneous pemphigus, and was never the only mucosal manifestation. The most common clinical signs were conjunctival hyperemia (87.5 %), erosions on the eyelids (41.6 %) as well as of the palpebral/bulbar conjunctiva (33.3 %) and at the medial epicanthus (20.8 %). The most relevant associated symptoms included local pain/stinging (71.4 %), irritation (47.6 %), photophobia (38.1 %), and epiphora (23.9 %). Ocular PV improved with systemic and adjuvant topical therapies. Only two patients experienced sequelae. CONCLUSIONS: In patients with PV, ocular involvement is an exception. Ocular PV is associated with greater disease activity, and usually follows a benign course. Sites affected are the conjunctiva, the eyelids, or both.
BACKGROUND AND OBJECTIVES: Ocular/periocular involvement in pemphigus vulgaris (OPV) has rarely been reported. The objective of the present study was to investigate the pattern of OPV and define the prognostic value of its manifestation. PATIENTS AND METHODS: From 1985 to 2014, a total of 167 patients with pemphigus vulgaris (PV) were treated at four tertiary Spanish hospitals. In this retrospective study, we included all patients with OPV. Clinical data and information on associated symptoms were obtained from patients' medical records. RESULTS: Only 24 (14.3 %) of all PV patients had ocular lesions. In most cases, -ocular involvement was preceded by PV lesions at various other sites (mean duration: 33.7 months). Ocular PV lesions occurred during flares of mucocutaneous pemphigus, and was never the only mucosal manifestation. The most common clinical signs were conjunctival hyperemia (87.5 %), erosions on the eyelids (41.6 %) as well as of the palpebral/bulbar conjunctiva (33.3 %) and at the medial epicanthus (20.8 %). The most relevant associated symptoms included local pain/stinging (71.4 %), irritation (47.6 %), photophobia (38.1 %), and epiphora (23.9 %). Ocular PV improved with systemic and adjuvant topical therapies. Only two patients experienced sequelae. CONCLUSIONS: In patients with PV, ocular involvement is an exception. Ocular PV is associated with greater disease activity, and usually follows a benign course. Sites affected are the conjunctiva, the eyelids, or both.
Authors: Brendon W H Lee; Jeremy C K Tan; Melissa Radjenovic; Minas T Coroneo; Dedee F Murrell Journal: Orphanet J Rare Dis Date: 2018-05-22 Impact factor: 4.123