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Grand Rounds Shivani V. Reddy, M.D. University of Louisville

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Presentation on theme: "Grand Rounds Shivani V. Reddy, M.D. University of Louisville"— Presentation transcript:

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 Exam 4→3 VA P EOM: full OU CVF: superior field limitation OU 20/20-1
20/ (PH: NI) 4→3 + 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 Pathology results
Same day: UL + LL epilation OS, aggressive lubrication OS cicatricial entropion 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 Cicatricial Pemphigoid

12 Ocular Cicatricial Pemphigoid
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 Cicatricial Pemphigoid
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 Cicatricial Pemphigoid
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 Cicatricial Pemphigoid
4 Stages Stage I – Chronic conjunctivitis with subepithelial fibrosis

16 Ocular Cicatricial Pemphigoid
4 Stages Stage II – Shortening of the inferior fornix

17 Ocular Cicatricial Pemphigoid
4 Stages Stage III – Symblepharon formation

18 Ocular Cicatricial Pemphigoid
4 Stages Stage IV – End stage disease manifesting as ankyloblepharon, severe sicca syndrome, severe ocular surface keratinization

19 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

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

21 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

22 Ocular Cicatricial Pemphigoid Immunoperoxidase Assay
Performed if immunofluorescence is negative but strong clinic suspicion Increases sensitivity of testing from %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

23 Ocular Cicatricial Pemphigoid
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

24 Ocular Cicatricial Pemphigoid
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

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26 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.

27 Thank You


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