Stevens Johnson Syndrome

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Stevens Johnson Syndrome : 

Stevens Johnson Syndrome

What is Stevens Johnson Syndrome? : 

What is Stevens Johnson Syndrome? May be called : Toxic Epidermal Necrolysis Syndrome Erythema Multiforme Major Discovered in 1922 by Drs. A.M. Stevens and S.C. Johnson, pediatricians Serious Inflammatory Disease Process!

Definition : 

Definition A severe, life threatening drug reaction Manifested by multiple lesions on skin, mucous membranes and conjunctiva May permeate to lungs and kidneys and other organs, increasing severity of illness.

Demographics : 

Demographics Can affect anyone More children than adults Fatal in 15% of cases Develops most often in winter and early spring

Causes : 

Causes Most commonly a reaction to any ingested drug Most common: Sulfas NSAIDS Anticonvulsants (Phenytoin, Valproic Acid, Phenobarbital) Vaccines (Tuberculin, Polio) May also be caused by infectious agent such as mycoplasma, Coxsackie Virus, Echovirus, Group A strep and herpes simplex Also associated with Leukemia and Lymphomas Many cases have no known cause

Symptoms : 

Symptoms Lesions in: the eyes, ears, skin, oral, anal and genital areas Redness of eyes and eyelids Recent History of causative drug use or illness Fatigue

Disease Course : 

Disease Course Usually begins with non specific viral illness symptoms High fever –may last 1 to 14 days Flu- like illness Malaise headache anorexia coughing General body aches burning sensation in eyes

Disease Course : 

Disease Course Rash develops 7 to 21 days after exposure to trigger. Begins as red, papular rash; then small red blisters on reddened or dusky base; progresses to characteristic bullae.

Disease course : 

Disease course Lesions of oral mucosa Lips, oral and pharyngeal mucous membranes May be so painful that affected individual does not eat

Disease course : 

Disease course Conjunctivitis Severe Often with purulent drainage

Disease Course : 

Disease Course Skin Lesions Begin as small open sores – may appear as “target” lesions May be painful and itchy .

Disease Course : 

Disease Course May look like blister like bullae

Disease process : 

Disease process Skin lesions begin on face, trunk, hands, soles of feet will continue to progress and spread

Disease process : 

Disease process As skin lesions continue and begin to spread skin may begin to slough off- Nikolsky’s sign

Disease process : 

Disease process

Disease Process : 

Disease Process Tissue sloughing

Disease Process : 

Disease Process Lesions will begin to crust over after 2-3 weeks. Some may become necrotic or erode Others may become hemorrhagic, especially at lips or gums.

Complications : 

Complications Pain from oral lesions leads to decreased food and fluid intake Dehydration Weight loss In children, may lead to oral aversion and need for enteral feeding

Complications : 

Complications If eyes are affected: May lead to permanent scarring of cornea Iritis Keratitis Adhesions of eyelids Vision Loss

Complications : 

Complications If lesions spread to trachea and lungs can lead to patchy infiltrates and pneumonias Can occlude respiratory tract requiring intubation If lesions spread to kidneys can lead to renal failure Superinfection of compromised skin

Diagnosis : 

Diagnosis Based on : History of antibiotic use or other known trigger symptoms, distribution and appearance of lesions May culture lesions May perform skin biopsy

Complications : 

Complications Skin erosion and necrosis requiring skin grafts Arthritis Arthralgia Myalgias

Risk of Mortality : 

Risk of Mortality 15 % of cases result in death. SCORTEN score –determines risk. 1 point for each of following criteria : Age>40 Diagnosis of Cancer Heart rate >120 10% skin detachment from bullae initially BUN >10 mmol/L Serum glucose level >14mmol/L Bicarbonate level <20mmol/L Holcomb,2007

Risk of Mortality : 

Risk of Mortality SCORTEN score continued Risk of death is based on score 0-1=3.2% or more 2=12/1% or more 3= 35% or more 4=58.3 % or more >5=90% or more Holcomb, 2007

Treatment : 

Treatment Identification and Removal of Cause Oral and Topical Steroids Comfort measures Viscous Lidocaine oral rinses Antihistamines for itching Meticulous Oral Hygiene and skin care

Treatment : 

Treatment May require ICU IV therapy Pain treatment- often Morphine If extremely severe or widespread – Burn unit

Treatment : 

Treatment If cause is from bacteria (mycoplasma) then antibiotic treatment If lesions are present in the eyes then Ophthalmology care and appropriate antibiotic eye treatment Hydration

Resources for more information : 

Resources for more information http://www.healthscout.com/ency/68/698/main.html www.sjsupport.org

References : 

References Holcomb, Susan S. Dodging the bullae: Stevens-Johnson Syndrome Nursing 2007 Vol 37, No. 4, April 2007 pp 64cc1-64cc3. Parillo,SJ and Parillo,CV: Stevens Johnson Syndrome http://www.emedicine.com last accessed 4/3/2007 Roujeau, Jean-Claude MD et al; Medication Use and the risk of Stevens Johnson Syndrome or Toxic Epidermal Necrosis New England Journal of Medicine ,Dec 14,1995 pp 1600-1608. Stern, Robert MD: Utilization of Hospital and Outpatient Care for adverse cutaneous reactions to medications Pharmacoepidemiology and Drug Safety (in press) accessed from www.wileyinterscience.wiley.com, 4/2/2007 http:healthscout.com/ency68/698.html last accessed 4/5/2007 www.sjsupport.org last accessed 4/6/2007