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Grand Rounds

Grand Rounds. Shivani V. Reddy, M.D. University of Louisville Department of Ophthalmology and Visual Sciences. History. CC : “eyelashes turned in”

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Grand Rounds

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  1. Grand Rounds Shivani V. Reddy, M.D. University of Louisville Department of Ophthalmology and Visual Sciences

  2. History • CC: “eyelashes turned in” • HPI: 72 y/o WM referred to oculoplastics clinic for a progressive trichiasis over 3-4 years. Patient states that growth is much more pronounced in the left eye . Also c/o chronic tearing, irritation and yellowish-white discharge in both eyes, worse on the left. States that overall symptoms have been progressing over about a 5 year span

  3. History POHx: Retinal detachment OS 1997, CE + IOL OU PMHx: Bullous Pemphigoid, Peripheral neuropathy, Asthma, Hypothyroidism, HTN FAMHx: noncontributory ROS:joint pain, muscle aches and difficulty swallowing intermittently MEDS:dapsone, zioptan, avodart, bystolic, cymbalta, nexium, b12, synthroid, NKDA

  4. 4→3 20/20-1 4→3 20/100+1 (PH: NI) Exam VA P + RAPD OS EOM: full OU CVF: superior field limitation OU

  5. Anterior Segment OD OS Lids/Lashes mild entropion UL+LL entropian few trichiatic lashes trichiasis Conj mild injection symblepharon 2+ injection Cornea multiple SPE multiple SPE inferonasal corneal erosion Iris WNL WNL Lens PCIOL PCIOL

  6. Physical Exam HENT: single tense vesicular lesion on soft palate Thorax: 2 vesicular lesions on upper back Extremities: single vesiculo-bullous lesion on right leg

  7. Skin Lesion erupted bullous lesion on the right lower extremity

  8. Summary 72 y/o WM presents with trichiasis OU 2/2 entropion, decreased visual acuity OS, symblepharon OS , 2+ conjunctival injection OS with an inferonasal corneal erosion. Dermatologic exam reveals vesicular lesions on the soft palate, upper back and lower extremity • DDx: • Autoimmune Cicatricial Conjunctivitis • MMP, Sarcoidosis , SLE, Lichen Planus, IgA dermatosis • Atopic Keratoconjunctivitis • Ocular Rosacea • Chronic Infectious Conjunctivitis • Adenovirus , streptococcus • Pseudopemphigoid (drug-induced ) • Conjunctival Trauma

  9. Treatment • Same day: UL + LL epilation OS, aggressive lubrication • OS cicatricialentropion repair + MMG of Upper and lower lid • Pathology results • Acutely inflamed tissue infiltrated with histiocytes, lymphocytes and neutrophils. Sub-epithelial fibrosis lacking elastic fibers indicative of scarring • Immunofixation not performed

  10. One Month Post-Op Visit • Grafts healing well, significant inflammation persistent, • no residual trichiasis

  11. Mucous Membrane Pemphigoid • Group of heterogeneous diseases characterized by inflammatory blistering of the oral, ocular, pharyngeal, laryngeal and genital mucosa • Main pathological feature: linear deposits of IgG, IgA and C3 in the epithelial basement membrane zone • When MMP presents as a chronic scarring conjunctivitis, it is known as Ocular CicatricialPemphigoid

  12. Ocular CicatricialPemphigoid Clinical features • Early on, signs of chronic or relapsing conjunctivitis with vesicles detected on the conjunctiva • Tearing , burning, mucous drainage • Loss of goblet cells • As the disease progresses, conjunctival shrinkage can cause impaired eye movements and lagophthalmos • Lid margin inflammation and scarring  trichiasis • Eventually trichiasis and gland loss lead to progressive corneal keratinization and scarring

  13. Ocular CicatricialPemphigoid Epidemiology • Incidence: 1/8000 – 1/46000 ophthalmic patients • Average age of diagnosis: 60 – 70 years • Female:Male: 1.5:1 – 3:1 • No geographic or racial predilection

  14. Ocular CicatricialPemphigoid Pathogenesis • Binding of circulating autoantibodies (IgG, IgA, C3 and other complement factors) to the BMZ (lamina lucida of the dermal- epidermal junction) • 205 kd β4 peptide of α6β4 integrin most frequent target • Why scarring instead of bullae formation? • Autoantibody binding to BMZ  secretion of cytokines (TNF-a, IL-1, migration inhibiting factor)  recruitment of inflammatory cells  release of pro-fibrotic cytokines such as TGF-beta and IFN-gamma  scarring • Inciting Event unknown

  15. Ocular CicatricialPemphigoid 4 Stages Stage II – Shortening of the inferior fornix

  16. Ocular CicatricialPemphigoid 4 Stages Stage III – Symblepharon formation

  17. Ocular CicatricialPemphigoid 4 Stages Stage IV – End stage disease manifesting as ankyloblepharon, severe sicca syndrome, severe ocular surface keratinization

  18. Ocular Cicatricial Pemphigoid Diagnosis • Most cases are caught in stage 2 to 3 and beyond due to the often insidious nature of progression • Diagnosis is based on: • Clinical Features • Tissue Biopsy • Should be performed perilesionally • Conj biopsy best during active disease • Specimen handling is extremely important as using the wrong specimen fixative can lead to false negative results

  19. Ocular Cicatricial Pemphigoid Hematoxylin & Eosin Staining • inflammatory infiltrate of variable intensity . Contains neutrophils, macrophages, plasma cells, lymphocytes, and Langerhans cells • Essentially nonspecific

  20. Ocular Cicatricial Pemphigoid Direct Immunofluorescence • Characteristic finding : Linear deposition of IgG, IgA, and/or C3 in basement membrane • However, diagnostic sensitivity is only around 50% . Therefore a negative result does not rule out a disease process

  21. Ocular Cicatricial Pemphigoid Immunoperoxidase Assay • Performed if immunofluorescence is negative but strong clinic suspicion • Increases sensitivity of testing from 52- 83%1 1. Power WJ, Neves RA, Rodriguez A, Dutt JE, Foster CS. Increasing the diagnostic yield of conjunctival biopsy in patients with suspected ocular cicatricial pemphigoid. Ophthalmology. 1995;102(8):1158

  22. Ocular CicatricialPemphigoid Treatment • Mild to Moderate Disease • Dapsone 50 – 200 mg/day for 12 weeks • Important to check labs – hemolytic anemia risk • MTX, mycophenolate, azathioprine can also be used, but more serious side effect profile • Severe Disease • Cyclophosphamide +/- Prednisone for 12 months or less • Must beware of leukopenia • Newer Therapies • IVIG • Rituximab

  23. Ocular CicatricialPemphigoid Treatment • Surgical Intervention • Entropion repair • Symblepharon excision • limbal stem cell transplantation, PK, keratoprosthesis • Maintainence Measures • Aggressive ocular lubrication • Lid hygiene for infection prevention • Epilation • PROGNOSIS? Current literature shows long term remission in 1/3 of patients for an average of 34 months with IM therapy

  24. References 1. BSCS Volume 8, External Diseases and Cornea . 2013 2. Pepose,Holland, Wilhelmus. Ocular Infection & Immunity. 1996 3. 1. Power WJ, Neves RA, Rodriguez A, Dutt JE, Foster CS. Increasing the diagnostic yield of conjunctival biopsy in patients with suspected ocular cicatricial pemphigoid. Ophthalmology. 1995;102(8):1158 4. Ahmed M, Zein G, Khawaja F, Foster CS. Ocular cicatricial pemphigoid: pathogenesis, diagnosis and treatment. Prog Retin Eye Res 2004; 23:579. 5.Fleming TE, Korman NJ. Cicatricial pemphigoid. J Am Acad Dermatol 2000; 43:571. 6. Foster CS. Cicatricial pemphigoid. Trans Am Ophthalmol Soc 1986; 84:527. 7. Chan LS, Ahmed AR, Anhalt GJ, et al. The first international consensus on mucous membrane pemphigoid: definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators. Arch Dermatol 2002; 138:370. 8.Letko E, Bhol K, Foster SC, Ahmed RA. Influence of intravenous immunoglobulin therapy on serum levels of anti-beta 4 antibodies in ocular cicatricial pemphigoid. A correlation with disease activity. A preliminary study. Curr Eye Res 2000; 21:646. 9. 60.Foster CS, Chang PY, Ahmed AR. Combination of rituximab and intravenous immunoglobulin for recalcitrant ocular cicatricial pemphigoid: a preliminary report. Ophthalmology 2010; 117:861.

  25. Thank You

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