Stevens-Johnson Syndrome

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    Stevens-Johnson Syndrome - Presentation Transcript

    1. Resident Clinical Conference Ying Pan, MD
    2. Case presentation
      • cc: sore throat and eye pain
      • HPI: 45 year old African American woman who presented with 2 days of worsening sore throat and bilateral eye pain and redness.
        • mild eye discharge, fever 103 F, malaise
      • PMH: Pneumonia 1990s
      • Toothache 2 weeks ago, prescribed medication by dentist
      • POH: none
      • PSH: none
      • NKDA
      • Meds: none
    3. Title Name
      • Content
    4. presentation
      • Content
    5. Title Name
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    6. Title Name
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    7. Exam
      • Visual acuity (near card): 20/40 OU
      • Pupils: round and reactive to light, no APD
      • Motility: grossly intact
      • Tp: 15 OD, 14 OS
    8. Title Name
      • Content
    9. Title Name
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    10.  
    11.  
    12. Epidermal Cell Apoptosis
      • Erythema Multiforme Minor
        • cutaneous involvement
      • Erythema Multiforme Major (Stevens-Johnson Syndrome)
        • widespread skin involvement, usually < 10% of BSA
        • at least two distinct mucosal sites (92-100% of cases)
      • Toxic Epidermal Necrolysis (TEN)
        • widespread skin involvement, usually > 30% of BSA
        • at least two distinct mucosal sites (almost 100% of cases)
    13. Etiologies
      • Medications (30-50% of SJS, 80% of TEN)
        • Antibiotics
        • Anticonvulsants
        • Cough/cold medications
        • NSAID
        • Anti-gout
        • Anti-psychotics
        • Carbonic anhydrase inhibitors
      • Infections (15% of SJS)
        • Herpes simplex virus
        • Adenovirus
        • Streptococci
        • Mycoplasma pneumonia
    14. Clinical Presentation
      • Prodrome
        • fever, flu-like symptoms, skin tenderness, photophobia
      • Signs and symptoms
        • Skin: ill defined, erythematous macular or palpular lesions with purpuric centers  vesicles and bullae  skin necrosis  sloughing of skin.  
        • Mucous membrane: mouth, conjunctiva, GI, respiratory tracts
        • Facial edema
        • Swelling of tongue
        • Sore throat
        • Shedding of nails
      • Incidence
        • SJS: 2-6 cases per million people per year
        • TEN: 0.4-1.2 cases per million people per year
      • Mean age
        • SJS: range from 25-47
        • TEN: range from 46-63
      • Mortality
        • SJS: 1-3 %
        • TEN: 10-50 %
        •  
      • Intervention
        • Supportive care
        • Steroids
        • Immunoglobulin
        • Plasmapheresis
    15. Acute Ocular Disease
      • Lid edema, episcleritis, conjunctivitis
      • Conjunctival bullae, ulceration
      • M embrane or pseudomembrane, adhesion/symblepharon
    16. Acute Ocular Management
        • Topical corticosteriods
        • Lubricating eye drops
        • Topical antibiotics
        • Mechanical lysis of adhesion
        • Scleral rings
        • Scleral contact lens
        • Amniotic membrane graft/patch
    17. Late Ocular Manifestations
      • Lid margin scarring
        • Entropion, Trichiasis
      • Punctal occlusion
      • Conjunctival scarring, keratinization, squamous metaplasia
        • Decrease or loss of mucus-secreting goblet cells
      • Fornix foreshortening, symblepharon, ankyloblepharon
      • Meibomian gland orifice metaplasia
      • Recurrent conjunctival inflammation
      • Cicatricial lid margin, tarsal and conjunctival changes strongly correlate with corneal complications
      • Keratopathy: recurrent or nonhealing epithelial defects, ulceration, neovascularization, limbal stem cell deficiency
    18. Chronic Ocular Complications
      • Ocular Surface Support
      • Perservative free artificial tears
      • Punctal occlusion
      • Autologous serum
      • Ocular surface protection
      • Gas-permeable scleral contact lenses
      • Retinoic Acid Ointment
      • Chronic Ocular Surface Inflammation
      • Topical steroids
      • Cyclosporine A
      • Systemic immunosuppressive
    19. Ocular surface reconstruction
      • Trichiasis repair
      • Entropion repair
      • Amniotic membrane transplantation
      • Mucous membrane graft
      • Limbal stem cell transplantation
        • Limbal stem grafts
        • Keratolimbal allograft (KLAL)
      • Penetrating keratoplasty
      • Keratoprosthesis
    20.  
    21. References
      • Romero-Rangel T et al. Gas-Permeable Scleral Contact Lens Therapy in Ocular Surface Disease. Am J Ophthalmol 2000; 130:25-32
      • Wall V et al. Management of the Late Ocular Sequelae of Stevens-Johnson Syndrome.
      • The Ocular Surface. 2003 (October), Vol 1, No. 4 192-200.
      • Khalili B. and Bahna S. Pathogenesis and Recent Therapeutic Trends in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. Ann Allergy Asthma immunol. 2006; 97:272-281.
      • Flach A, Smith RE, Fraunfelder FT. Stevens-Johnson Syndrome Associated with Methazolamide Treatment Reported in Two Japanese-American Women. Ophthalmology 1995; 102:1677-1680.
      • Sall K, Stevenson OD, Mundorf TK et al. Two Multicenter, Randomized Studies of the Efficacy and Safety of Cyclosporine Ophthalmic Emulsion in Moderate to Severe Dry Eye Disease. Ophthalmology 2000; 107: 631-639.
      • Tsubota K et al. Treatment of Persistent Corneal Epithelial Defect by Autologous Serum Application. Ophthalmology 1999; 106: 1984-1989.
      • Inatomi T. et al. Ocular Surface Reconstruction with Combination of Cultivated Autologous Oral Mucosal Epithelial Transplantation and Penetrating Keratoplasty. Am J Ophthalmol 2006; 142:757-764.
      • Power et al. Analysis of the Acute Ophthalmic Manifestation of the Erythema Multiforme/Stevens-Johnson Syndrome/Toxic EpidermalNecrolysis Disease Spectrum. Ophthalmology 1995; 102:1669-1676.
      • Kaido M et al. Punctal Occlusion in the Management of Chronic Stevens-Johnson Syndrome. Ophthalmology 2004; 111:895-900.
      • Singer L et al. Vitamin A in Stevens-Johnson Syndrome. Ann Ophthalmol 1989; 209-210

    + Ryan AlfonsoRyan Alfonso, 2 years ago

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