Omeed Memar1, Sayena Jabbehdari2, Benjamin Caughlin3, Ali R Djalilian2. 1. Academic Dermatology & Skin Cancer Institute, Chicago, IL, USA. Electronic address: omeedmemar@gmail.com. 2. Department of Ophthalmology, University of Illinois Health Hospital System, Chicago, IL, USA. 3. Department of Surgery / Division of Otolaryngology, John H. Stroger, Jr. Hospital of Cook County, Chicago, IL, USA; Division of Facial Plastic and Reconstructive Surgery, Jesse Brown VAMC, Chicago, IL, USA; Division of Facial Plastic and Reconstructive Surgery, University of Illinois Health Hospital System, Chicago, IL, USA; Kovak Cosmetic Center, Oakbrook Terrace, Illinois, USA.
Abstract
PURPOSE: A review of the published literature on the history, pathogenesis, and treatment of pemphigus vulgaris (PV) and its ocular involvement. METHODS: Literature searches were conducted in MEDLINE (Ovid), and google scholar for pemphigus vulgaris and ocular PV. Inclusion criteria were given to meta-analysis, case-controlled studies, and documented case reports. The data were examined and independently analyzed by more than two of the authors. RESULTS: PV is a humoral autoimmune disease with a preponderance of IgG4 anti-desmoglein 3 antibodies. Upon antibody binding, there is an intracellular signaling mechanism that leads to blister formation. Ocular findings are seen in up to 16% of PV patients with conjunctivitis being the most common clinical presentation. New steroid-sparing agents have helped with the control of this deadly disease, and with better understanding of the pathogenesis of PV, other cytokine blockers currently available are promising steroid-sparing agents. CONCLUSIONS: Ocular pemphigus can occasionally present prior to mucocutaneous findings. Recalcitrant conjunctivitis with conjunctival blisters should warrant a workup for systemic PV.
PURPOSE: A review of the published literature on the history, pathogenesis, and treatment of pemphigus vulgaris (PV) and its ocular involvement. METHODS: Literature searches were conducted in MEDLINE (Ovid), and google scholar for pemphigus vulgaris and ocular PV. Inclusion criteria were given to meta-analysis, case-controlled studies, and documented case reports. The data were examined and independently analyzed by more than two of the authors. RESULTS:PV is a humoral autoimmune disease with a preponderance of IgG4 anti-desmoglein 3 antibodies. Upon antibody binding, there is an intracellular signaling mechanism that leads to blister formation. Ocular findings are seen in up to 16% of PVpatients with conjunctivitis being the most common clinical presentation. New steroid-sparing agents have helped with the control of this deadly disease, and with better understanding of the pathogenesis of PV, other cytokine blockers currently available are promising steroid-sparing agents. CONCLUSIONS: Ocular pemphigus can occasionally present prior to mucocutaneous findings. Recalcitrant conjunctivitis with conjunctival blisters should warrant a workup for systemic PV.