Stevens-Johnson Syndrome

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  • 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
    • Content
  • 6. Title Name
    • Content
  • 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
    • Content
  • 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
  • 15.
    • 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
  • 16. Acute Ocular Disease
    • Lid edema, episcleritis, conjunctivitis
    • Conjunctival bullae, ulceration
    • M embrane or pseudomembrane, adhesion/symblepharon
  • 17. Acute Ocular Management
      • Topical corticosteriods
      • Lubricating eye drops
      • Topical antibiotics
      • Mechanical lysis of adhesion
      • Scleral rings
      • Scleral contact lens
      • Amniotic membrane graft/patch
  • 18. 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
  • 19.
    • Cicatricial lid margin, tarsal and conjunctival changes strongly correlate with corneal complications
    • Keratopathy: recurrent or nonhealing epithelial defects, ulceration, neovascularization, limbal stem cell deficiency
  • 20. 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
  • 21. 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
  • 22.  
  • 23. 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