This issue of eMedRef provides information to clinicians on the pathophysiology, diagnosis, and therapeutics of Stevens-Johnson Syndrome

Bhatt, Ami

Carlson, Nathan

English

University of Missouri: MOspace

Stevens-Johnson Syndrome Page 1 of 4    8.17.12  STEVENS-JOHNSON SYNDROME   Background   1. Definition  o Epidermal detachment of <10% of body surface area 1  o Toxic Epidermal Necrolysis (TEN): >30% of body surface area involvement o Mucous membrane involvement: oral, genital, anal, nasal, conjunctival  Pathophysiology    1. Pathology of Disease o Drug hypersensitivity leads to Major Histocompatibility Complex-I (MHC-1) restricted drug presentation and is followed by an expansion of cytotoxic T-lymphocytes leading to infiltration of skin lesions with cytotoxic T-lymphocytes and Null Killer (NK) cells. o Keratinocyte apoptosis due to Fas and Fas ligand (CD95)1  o Reaction to underlying infection 2. Incidence, Prevalence o 1 to 7 cases per million person-years  3. Risk Factors: o 74-94% of cases triggered by preceding medication or URI infection 1  Trimethoprim-sulfamethoxazole and other sulfonamides most common.  Less common: cephalosporin, quinolones, aminopenicillins   Cross reactivity among beta lactams and cephalosporins o Myscoplasma pneumonia, HIV, herpesvirus - more common in Erythema Multiform, hepatitis A o HLA-B1502 gene presence in Chinese patients with use of carbamazepine 1 4. Morbidity / Mortality o 1-3% a year o Less in children then adults o Sepsis major cause of death  Diagnostics  1. History o Fever and influenza like symptoms, occurring 1-3 weeks after use of offending agent o Rash o Erosive changes on the lips, mouth, conjunctivae, genital area 2. Physical Examination o 90%  of cases involve mucous membranes, including eyes, mouth, nose and genitalia o Skin lesions: generalized macules with purpuric centers.                                                        Stevens-Johnson Syndrome Page 2 of 4    8.17.12   Macules progress to large blisters which then cause epidermal detachment.   3-5 days later separation of epidermis progresses o Ulcerative stomatitis o Labored breathing if tracheobronchial involvement o Large wound areas cause pain, protein/ fluid loss, bleeding o Nikolsky sign: light pressure causing detachment of full thickness epidermis o Hyper-/hypo-pigmentation of skin o Esophageal ulceration o Ocular: swollen, erythematous eyelids, conjunctivitis, keratitis, endophthalmitis; can cause loss of vision 3. Diagnostic Testing o Skin biopsy: shows separation of epidermis at the dermal-epidermal junction.  o Mycoplasma PCR, IgG, IgM 4. Laboratory evaluation o Bun, creatinine, lytes, CBC, glucose, skin swabs for erosions, blood cultures 5. Diagnostic imaging o CXR 6. Other studies o Direct immunofluorescence  Differential Diagnosis  1. Key Differential Diagnoses o Erythema multiforme o Bullous pemphigoid o Staphylococcal scalded skin syndrome o Linear IgA dermatosis o Behcet’s syndrome o Kawasaki disease o Toxic shock syndrome o Toxic epidermal necrolysis >30% of body involvement  Therapeutics  1. Acute Treatment o Immediate drug discontinuation o Transfer to burn unit improves mortality1 o 600 calories or 22% less per day than burn patients 1 o Provide analgesia: swish and swallow o Prevent dehydration; monitor lytes o Mycoplasma treatment: 3   Macrolides  Macrolide resistance common: fluoroquinolones can be used or tetracyclines, but use of either not currently recommended for children                                                  1   3  Stevens-Johnson Syndrome Page 3 of 4    8.17.12  o Skin lesion treatment with copper sulfate, silver nitrate or sulfadiazine cream (can cause cross reactivity if a sulfa was predisposing drug) 1  Acticoat, Aquacel Ag, supathel: synthetic materials containing nanocrystalline silver; can be changed weekly 1  Bland emollients better choice 5 o Avoid skin trauma o Corticosteroid treatment: shorter duration of illness 1  Some showed slower time to recovery  May cause underlying infection to flare  Can have more medical complications, administering steroids for greater than 48 hours showed high rate of infection 1  No consensus reached for treatment, but trend - more complications without significant benefit…esp. >48 hrs Tx o Corticosteroids vs IVIG vs supportive care: no significant effect on mortality 1 o IVIG: in severe cases can prevent further skin manifestations o Plasmapharesis can be used safely for extensive cases 2. Further Management (24 hrs) o Ocular  Long term steroid treatment for ocular complications 1  Pulsed topical steroid treatment for 4 days (In adults, 2mg/kg per day)  minimal ocular manifestations,  o Note: if significant skin sloughing has already occurred, then would cause more systemic complications   Lubrication of eyes  Amniotic membrane transplantation 2  Must occur within 10 days of disease  Used in severe cases of ocular involvement: large areas of epithelial sloughing of conjunctiva, cornea and lid margins  Used alone (without steroids) show most benefit  Used locally, so no systemic effects 3. Long-Term Care o Chronic ophthalmic complications: corneal inflammation1  30% in surviving children   Up to 74% in adults  Severe in 25%  Follow-Up   1. Return to Office o 2-3 days for close follow-up                                                          Stevens-Johnson Syndrome Page 4 of 4    8.17.12  o If clinical symptoms worsen then seek immediate medical attention 2. Refer to Specialist o Dermatology: excessive skin peeling, ulcerations, alopecia, lack of nail growth o Ophthalmology:  Ocular manifestations; photophobia, keratitis, corneal abrasions, visual impairment and dry eye. 3. Admit to Hospital o Severe skin sloughing greater then 10 percent of body surface, ocular manifestations, respiratory compromise  Prognosis 1. Recurrence rate: 20% of affected children up to 7 years after first episode 4 2. Long term ocular complications  Prevention  1. Avoid eliciting drugs/medications  Patient Education   1. http://www.mayoclinic.com/health/stevens-johnson-syndrome/DS00940 2. http://www.sjsupport.org/ 3. http://www.skinassn.org/what-is-stevens-johnson-syndrome.html  References  1. Gerull R, Nelle M, Schaible T. Toxic Epidermal Necrolysis and Stevens Johnson Syndrome: A Review. Critical Care Medicine 2011; 39: 6 2. Gregory DG. Treatment of Acute Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Using Amniotic Membrane: A review of 10 Consecutive Cases. Opthalmology 2011; 118: 908-914. 3. Atkinson PT, Boppana S, Theos A, Clements LS, Xiao L, Waites K. Stevens Johnson Syndrome in a Boy with Macrolide Resistant Myscoplasma Pnuemonia. Pediatrics 2011; 127: e1605-1609 4. Finkelstein Y, Soon GS, Acuna P, George M, Pope M, Ito S, Shear NH, Koren G, Shannon MW, Bournissen F. Recurrence and Outcomes of Stevens Johnson Syndrome and Toxic Epidermal Necrolysis in Children. Pediatrics 2011; 128: 723-728 5. Treat J. Stevens Johnson Syndrome and Toxic Epidermal Necrolysis. Pediatric Anals 2010; 39:10   Authors:  Ami Bhatt, MD, & Nathan D. Carlson, MD  CA - Kaiser Permanente Fontana FMRP   Editor:  Robert Marshall, MD, MPH, MISM, CMIO,  Madigan Army Medical Center, Tacoma, WA                                                    

Stevens-Johnson Syndrome

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Stevens-Johnson Syndrome

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