Grand Rounds - PowerPoint PPT Presentation

Loading...

PPT – Grand Rounds PowerPoint presentation | free to download - id: 6a350b-OGNiY



Loading


The Adobe Flash plugin is needed to view this content

Get the plugin now

View by Category
About This Presentation
Title:

Grand Rounds

Description:

Grand Rounds Shivani V. Reddy, M.D. University of Louisville Department of Ophthalmology and Visual Sciences * * * History CC: eyelashes turned in HPI: 72 y/o ... – PowerPoint PPT presentation

Number of Views:3
Avg rating:3.0/5.0
Slides: 27
Provided by: residents
Category:

less

Write a Comment
User Comments (0)
Transcript and Presenter's Notes

Title: Grand Rounds


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
VA
P
RAPD OS
EOM full OU CVF superior field limitation OU
5
Anterior Segment

OD
OS Lids/Lashes
mild entropion
ULLL 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 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
  • FemaleMale 1.51 31
  • 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 ß4 peptide of a6ß4 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 52- 831

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.
1995102(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

25
(No Transcript)
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. 1995102(8)1158 4.
Ahmed M, Zein G, Khawaja F, Foster CS. Ocular
cicatricial pemphigoid pathogenesis, diagnosis
and treatment. Prog Retin Eye Res 2004
23579. 5.Fleming TE, Korman NJ. Cicatricial
pemphigoid. J Am Acad Dermatol 2000 43571. 6.
Foster CS. Cicatricial pemphigoid. Trans Am
Ophthalmol Soc 1986 84527. 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 138370. 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 21646. 9. 60.Foster CS, Chang PY,
Ahmed AR. Combination of rituximab and
intravenous immunoglobulin for recalcitrant
ocular cicatricial pemphigoid a preliminary
report. Ophthalmology 2010 117861.
27
Thank You
About PowerShow.com