Abstract
Purpose
To describe the clinical characteristics of ocular involvement in patients with pemphigus at an ophthalmological referral center.
Methods
A retrospective review was conducted on patients with the immunopathological diagnosis of pemphigus examined between 1 January 2000 and 1 April 2010. Uncorrected distance visual acuity (UDVA), best corrected distance visual acuity (BCVA), ocular symptoms, and ocular surface inflammatory and scarring changes were assessed.
Results
A total of 15 patients were identified, with a mean age of 68.27±14.35 years, and 80% (n=12) were female. Extraocular involvement was reported in one patient. All of the eyes showed cicatricial changes in the conjunctiva. In all, 6 eyes (20%) were classified as stage I; 12 eyes (40%) as stage II; 10 eyes (33%) as stage III; and 2 eyes (7%) as stage IV. A statistically significant association was found between BCVA and the severity of ocular involvement. The mean BCVA logMAR was 1.66 (20/914), with a range from logMAR 0 (20/20) to logMAR 4 (NLP). Other ocular diseases were found in 8 (53.3%), systemic diseases in 10 (66.7%), and the use of pemphigus-inducing drugs in 10 patients (66.7%).
Conclusions
The present report represents the largest series of ocular involvement in pemphigus confirmed by immunopathology. The clinical manifestations varied from conjunctival hyperemia to corneal scarring and perforation. There was a strong association between scarring changes and low BCVA. Ocular and systemic diseases as well as the use of pemphigus-inducing drugs may predispose to ocular cicatricial changes observed in this series.
Similar content being viewed by others
Log in or create a free account to read this content
Gain free access to this article, as well as selected content from this journal and more on nature.com
or
References
Jordan E, Scott BS, Razzaque AA . The blistering diseases. Med Clin North Am 1998; 82: 1239–1283.
Simon DG, Krutchkoff D, Kaslow RA, Zarbo R . Pemphigus in Hartford County, Connecticut, from 1972 to 1977. Arch Dermatol 1980; 116: 1035–1037.
Stanley JR, Amagai M . Pemphigus, bullous impetigo, and the staphylococcal scalded-skin syndrome. N Engl J Med 2006; 355–1800.
Becker BA, Gaspari AA . Pemphigus vulgaris and vegetans: bullous diseases. Dermatol Clin 1993; 11: 429–452.
Burnham TK . Indirect cutaneous immunofluorescence. J Clin Pathol 1973; 26: 268–272.
Daoud YJ, Cervantes R, Foster CS, Ahmed AR . Ocular pemphigus. J Am Acad Dermatol 2005; 53: 585–590.
Palleschi GM, Giomi B, Fabbriet P . Ocular involvement in pemphigus. Am J Ophthalmol 2007; 144: 149–152.
Smith RJ, Manche EE, Mondino BJ . Ocular cicatricial pemphigoid and ocular manifestations of pemphigus vulgaris. Int Ophthalmol Clin 1997; 37: 63–75.
Hodak E, Kremer I, David M, Hazaz B, Rothem A, Feuerman P et al. Conjunctival involvement in pemphigus vulgaris: a clinical, histopathological and immunofluorescence study. Br J Dermatol 1990; 123: 615–620.
Lifshitz T, Levy J, Cagnano E, Halevy S . Severe conjunctival and eyelid involvement in pemphigus vulgaris. Int Ophthalmol 2004; 25: 73–74.
Baykal HE, Pleyer U, Sonnichsen K, Thiel HJ, Zierhut M . Severe eye involvement in pemphigus vulgaris. Ophthalmologe 1995; 92: 854–857.
Suami M, Kato M, Koide K, Usami Y, Hata N, Machida H et al. Keratolysis in a patient with pemphigus vulgaris. Br J Ophthalmol 2001; 85: 1263.
Foster CS, Wilson LA, Elkins LB . Immunosuppressive therapy for progressive ocular cicatricial pemphigoid. Ophthalmology 1982; 89: 340–353.
Huilgol SC, Black MM . Management of the immunobullous disorders II Pemphigus. Clin Exp Dermatol 1995; 20: 283–293.
Laforest C, Huilgol SC, Casson R, Selva D, Leibovitch I . Autoimmune bullous. Ocular manifestations and management. Drugs 2005; 65 (13): 1767–1779.
Korman N . Pemphigus. J Am Acad Dermatol 1988; 18: 1219.
Camisa C, Meisler DM . Immunobullous diseases with ocular involvement. Dermatol Clin 1992; 10: 555–570.
Merchant S, Weinstein M . Pemphigus vulgaris: the eyes have it. Pediatrics 2003; 112: 183–185.
Bean SF, Holubar K, Gillett RB . Pemphigus involving the eyes. Arch Dermatol 1975; 111: 1484–1486.
Bedell AJ . Ocular pemphigus: a clinical presentation of kodachromes. Trans Am Ophthalmol Soc 1964; 62: 109–122.
Bystryn JC, Rudolph JL . Pemphigus. Lancet 2005; 366: 61–73.
Yeh SW, Ahmed B, Sami N, Razzaque Ahmed A . Blistering disorders: diagnosis and treatment. Dermatol Ther 2003; 16: 214–223.
Falk ES . Pemphigus foliaceus in a patient with rhematoid arthritis and Sjogrens syndrome. Dermatologica 1979; 158: 348–354.
Diaz LA, Glamb RW, Silva Jr J . A syndrome of multiple immune autoreactivity. Arch Dermatol 1980; 116: 77–79.
Gholizadeh N, Zenouz A, Eslami H . Pemphigus vulgaris associated with rheumatoid arthritis in a patient no taking penicillamine. JODD 2012; 6 (1): 33–35.
Levine L, Bernstein JE, Soltani K, Medenica MM, Yung CW . Coexisting childhood pemphigus foliaceus and Graves disease. Arch Dermatol 1982; 118: 602–604.
Wolf R, Feuerman EJ . Pemphigus in association with autoimmune thyroid disease. Cutis 1981; 27: 423–424.
Leonhardt J, Heymann W . Thyroid disease and the skin. Dermatol Clin 2002; 20: 473–481.
Bartalena L, Bogazzi F, Chiovato L, Tanda ML, Martino E . Graves’ hyperthyroidism and ophthalmopathy associated with pemphigus vulgaris: onset of thyroid autoimmune disease during chronic low-dose glucocorticoid therapy. J Endocrinol Invest 1997; 20: 155–157.
Buhac J, Bhol K, Padilla Jr T, Foster CS, Ahmed AR . Coexistence of pemphigus vulgaris and ocular cicatricial pemphigoid. J Am Acad Dermatol 1996; 34 (5 Pt 2): 884–886.
Sami N, Bhol KC, Beutner EH, Plunkett RW, Leiferman KM, Foster CS et al. Simultaneous presence of mucous membrane pemphigoid and pemphigus vulgaris: molecular characterization of both autoantibodies. Clin Immunol 2001; 100 (2): 219–227.
Brenner S, Ruocco V . D-penicillamine-induced pemphigus foliaceus with autoantibodies to desmoglein-1. J Am Acad Dermatol 1998; 39: 137–138.
Brenner S, Bialy-Golan A, Crost N . Dipyrone in the induction of pemphigus. J Am Acad Dermatol 1997; 36: 488–490.
Matz H, Bialy-Golan A, Brenner S . Diclofenac: a new trigger of pemphigus vulgaris? Dermatology 1997; 195: 48–49.
Brenner S, Ruocco V, Bialy-Golan A, Tur E, Flaminio C, Ruocco E et al. Pemphigus and pemphigoid-like effects of nifedipine on in vitro cultured normal human skin explants. Int J Dermatol 1999; 38: 36–40.
Goldberg I, Kashman Y, Brenner S . The induction of pemphigus by phenol drugs. Int J Dermatol 1999; 38: 888–892.
Kaplan RP, Potter TS, Fox JN . Drug-induced pemphigus related to angiotensin-converting enzyme inhibitors. J Am Acad Dermatol 1992; 26: 364–366.
Goldberg I, Sasson A, Gat A, Srebrnik A, Brenner S . Pemphigus vulgaris triggered by glibenclamide and cilazapril. Acta Dermatovenerol Croat 2005; 13: 153–155.
Brenner S, Goldberg I . Drug-induced pemphigus. Clin Dermatol. 2011; 29: 455–457.
Ruocco V, De Angelis E, Lombardi ML . Drug-induced pemphigus. II. Pathomechanisms and experimental investigations. Clin Dermatol 1993; 11: 507–513.
Bae YI, Yun SJ, Lee SC, Park GT, Lee JB . Pemphigus foliaceus induced by an angiotensin II receptor blocker. Clin Exp Dermatol 2008; 33 (6): 721–723.
Newby CS, Barr RM, Greaves MW, Mallet AI . Cytokine release and cytotoxicity in human keratinocytes and fibroblasts induce d by phenols and sodium dodecyl sulfate. J Invest Dermatol 2000; 115: 292–298.
Feliciani C, Toto P, Amerio P, Pour SM, Coscione G, Shivji G et al. In vitro and in vivo expression of interleukin-1α and tumor necrosis factor-α mRNA in pemphigus vulgaris: interleukin-1α and tumor necrosis factor-α are involved in acantholysis. J Invest Dermatol 2000; 114: 71–77.
Ruocco V, Ruocco E . Pemphigus and environmental factors. G Ital Dermatol Venereol 2003; 138: 299–309.
Acknowledgements
We would like to thank C Guadalupe Quintero for her assistance with pathologic specimens, and Dr Mark Mannis for his critical review of the paper.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Competing interests
The authors declare no conflict of interest.
Rights and permissions
About this article
Cite this article
Chirinos-Saldaña, P., Zuñiga-Gonzalez, I., Hernandez-Camarena, J. et al. Cicatricial changes in ocular pemphigus. Eye 28, 459–465 (2014). https://doi.org/10.1038/eye.2014.3
Received:
Accepted:
Published:
Issue date:
DOI: https://doi.org/10.1038/eye.2014.3
Keywords
This article is cited by
-
Ocular Mucous Membrane Pemphigoid: Current State of Pathophysiology, Diagnostics and Treatment
Ophthalmology and Therapy (2019)
-
A review of scoring systems for ocular involvement in chronic cutaneous bullous diseases
Orphanet Journal of Rare Diseases (2018)
-
The 2016 Bowman Lecture Conjunctival curses: scarring conjunctivitis 30 years on
Eye (2017)


