Transcript Ocular Cicatricial Pemphigoid - University of Louisville Ophthalmology

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
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NIDDM for 7 years, HTN, hyperlipidemia
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Meds: Metformin, Januvia, Farxiga, Glimepiride,
Lisinopril, Lipofen, Lovastatin, ASA
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SxHx: Tonsillectomy, Tubal ligation,
Hysterectomy, Lumpectomy (breast)
Past Medical History
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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
32
Full
OS
20/25+
No rAPD
32
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
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Cicatricial conjunctivitis
Ocular cicatricial pemphigoid
 Infectious: trachoma
 Inflammatory: rosacea
 Autoimmune: linear IgA disease, Graft Versus Host
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Disease (GVHD), and Stevens Johnson Syndrome (SJS)
Allergic: atopy
 Conjunctival trauma, chemical burns
 Medicamentosa
 Radiation
 Neoplasia
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Medicamentosa Pseudopemphigoid
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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.
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Assessment
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54 yo female with bilateral upper eyelid
cicatricial entropion
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Plan
Bilateral upper eyelid cicatricial entropion repair
 Mucous membrane graft from lower lip
 Conjunctival biopsy from left lower lid
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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)
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Subtype of Mucous Membrane
Pemphigoid (MMP)
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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
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Age of onset 60-70 years
Usually presents in stage III
Female preponderance, 2:1
All races are affected
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Disease progresses in 75% of untreated pts
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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
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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