Stevens-Johnson Syndrome

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Stevens-Johnson Syndrome

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

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