Ocular involvement in pemphigus vulgaris - a retrospective study of a large Spanish cohort.
OBJECTIVE
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.


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.
Department of Dermatology, Unit of Autoimmune Blistering Skin Diseases; University Clinic of Navarra, School of Medicine, Pamplona, Navarra, Spain.
Agustin
A
España
Hospital Clinic of Barcelona, Spain.
Pilar
P
Iranzo
Hospital del Mar of Barcelona, Spain.
Josep
J
Herrero-González
Hospital Clinic of Barcelona, Spain.
José M
JM
Mascaro
Hospital Gregorio Marañon of Madrid, Spain.
Ricardo
R
Suárez
D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D015331:Cohort Studies; D015897:Comorbidity; D003229:Conjunctival Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology*; D005141:Eyelid Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology*; D015995:Prevalence; D012189:Retrospective Studies; D012307:Risk Factors; D013030:Spain / Q000453:epidemiology; D016896:Treatment Outcome
D000893:Anti-Inflammatory Agents