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Grand Rounds Conference Juan P. Fernandez de Castro, MD University of Louisville Department of Ophthalmology and Visual Sciences June 17, 2016 Subjective Referred by outside ophthalmologist for trichiasis CC: Eyelashes growing towards the eyes HPI: 54 yo female with a 1.5 years complaint of misdirected eyelashes that has gotten progressively worse. She used to epilate them weekly, now epilating every other day. Past Medical History NIDDM for 7 years, HTN, hyperlipidemia Meds: Metformin, Januvia, Farxiga, Glimepiride, Lisinopril, Lipofen, Lovastatin, ASA SxHx: Tonsillectomy, Tubal ligation, Hysterectomy, Lumpectomy (breast) Past Medical History Allergy: Morphine Former smoker (quit 10 years ago) RoS Blurred vision, dryness, mucous discharge, redness, itching, burning, tearing, droopy eyelids Objective VA (sc): Pupils: IOP: EOM: OD 20/20 32 Full OS 20/25+ No rAPD 32 Refused by pt Full SLE: Lids Conj Cornea A/C Iris Lens Vitreous Objective Bilateral upper eyelid cicatricial entropion with conjunctival scarring trichiasis Fibrous bands OS>OD Contact lens in place OU Deep and Quiet OU WNL OU Clear OU Clear OU Clinical Photos Differential Diagnosis Cicatricial conjunctivitis Ocular cicatricial pemphigoid Infectious: trachoma Inflammatory: rosacea Autoimmune: linear IgA disease, Graft Versus Host Disease (GVHD), and Stevens Johnson Syndrome (SJS) Allergic: atopy Conjunctival trauma, chemical burns Medicamentosa Radiation Neoplasia Medicamentosa Pseudopemphigoid Identical to OCP but is caused by the long-term use of certain topical medications. Resolution with discontinuation of the offending agent is diagnostic. Pilocarpine, epinephrine, timolol, idoxuridine, echothiophate iodide, and demecarium bromide. Assessment 54 yo female with bilateral upper eyelid cicatricial entropion Plan Bilateral upper eyelid cicatricial entropion repair Mucous membrane graft from lower lip Conjunctival biopsy from left lower lid Pathology 40x Soon Bahrami MD Section Chief and Asst Prof of Dermatopathology Dept of Medicine, Division of Dermatology Dept of Pathology & Laboratory Medicine University of Louisville Pathology 200x Immunofluorescence Ocular Cicatricial Pemphigoid (OCP) Subtype of Mucous Membrane Pemphigoid (MMP) Autoimmune conjunctivitis with autoantibodies against distinct structural components of the dermal–epidermal junction with subsequent cicatrization Pemphigoid diseases Pemphigoid diseases Enno Schmidt, Detlef Zillikens Lancet 2013; 381: 320–332 Bullous Pemphigoid Pemphigoid diseases Enno Schmidt, Detlef Zillikens Lancet 2013; 381: 320–332 Other Pemphigoids Pemphigoid diseases Enno Schmidt, Detlef Zillikens Lancet 2013; 381: 320–332 Stages Stage I – Chronic conjunctivitis with subepithelial fibrosis Stage III – Symblepharon formation Stage II – Shortening of the inferior fornix Stage IV – End stage ankyloblepharon, severe sicca syndrome, and keratinization Epidemiology Age of onset 60-70 years Usually presents in stage III Female preponderance, 2:1 All races are affected Disease progresses in 75% of untreated pts Systemic treatment is necessary Step Ladder Approach IVIg Failed treatment, or severe complications Severe Etanercept Infliximab Pentoxifylline Cyclophosphamide First line when vision loss is imminent Low-dose, pulsed regimen for elderly Mycophenolate mofetil (MMF) Cellcept Moderate Mild to Moderate Fewer side effects First line in pts without sight threatening complications Methotrexate Dapsone or Azathioprine High rates discontinuation Sulfapyridine Sulfasalazine Lower adverse effects Topical and Subconj Steroids Temporary Calcineurin inhibitors: (Cyclosporine, Tacrolimus) Alkilating agents: Mitomycin C in treatment after surgical lysis Future Directions Identification of the "susceptibility gene" and the target antigen Cloning the gene for that antigen, and develop a diagnostic test Re-educate the patient's white blood cells