Ocular involvement in pemphigus vulgaris – a retrospective study of a large Spanish cohort

• DOI: 10.1111/ddg.13221
• Publication Date: 2017-03-24T06:36:59Z
• Authors: Agustin España; Pilar Iranzo; Josep Herrero‐Gon...; José M. Mascaro; Ricardo Suárez
• Keywords: Male; Anti-Inflammatory Agents; Comorbidity; Middle Aged; Conjunctival Diseases; 3. Good health; Cohort Studies; 03 medical and health sciences; Treatment Outcome; 0302 clinical medicine; Risk Factors; Spain; Eyelid Diseases; Prevalence; Humans; Female; Pemphigus; Retrospective Studies

SummaryBackground and objectivesOcular/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 methodsFrom 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.ResultsOnly 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.ConclusionsIn 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.