Stevens Johnson Syndrome

From Anvita Health Wiki

Contents 1 Etiology 2 Epidemiology 3 Pathology 4 Genetics 5 Clinical-manifestations 6 Laboratory 7 Complications 8 Differential-diagnosis 9 Management 10 More General Terms 11 Additional Terms 12 References

Etiology

• hypersensitivity (most likely)

• possibly immunologic
• drugs & infection most likely triggers

• pharmaceutical agents

• antibiotics
• anticonvulsants
• see pharmaceutical agents causing SCARs

• infections

• Herpes simplex
• Mycoplasma

Epidemiology

• most common in young adults
• male:female ratio 2:1

Pathology

• acute epidermal necrosis

Genetics

• drug-induced SJS ( carbamazepine, phenytoin) linked to HLA-B*1502 in Han Chines (Asians, South Asian Indians)

Clinical-manifestations

• prodrome of fever, malaise, arthralgia, headache, respiratory symptoms, vomiting &/or diarrhea 1-14 days before appearance of skin lesions
• skin & mucous membrane manifestations

• diffuse pruritis or burning may occur in the prodromal phase
• early lesions are pink, edematous papules
• these then evolve into dull, red macules with central cyanosis or into vesicles (classic 'target lesions')
• lesions can coalesce & progress to flaccid bullae
• lesions are most prominent on extremities, palms & soles
• erosive lesions may occur on mucous membranes
• mucosa alone may be affected
• hemorrhagic crusting of oral mucosa is characteristic [2]

• systemic manifestations

• arthralgia
• hepatitis
• myocarditis
• nephritis
• pneumonia

• time course: 4-6 weeks

Laboratory

• blood cultures
• complete blood count ( CBC)

• anemia
• leukocytosis
• eosinophilia

• serum electrolytes, serum urea nitrogen, serum creatinine
• liver function tests
• erythrocyte sedimentation rate may be elevated
• urinalysis

• proteinuria
• hematuria

• skin biopsy if diagnosis uncertain

Complications

• infection
• mortality is 1-5%

Differential-diagnosis

• erythema multiforme
• toxic epidermal necrolysis ( TEN) is a severe form of SJS

• in SJS, skin detachment effects < 10% of body area
• in TEN, skin detachment effects > 30% of body area

Management

• hospitalize; admit to intensive care unit ( burn unit)
• identify & eliminate triggering agent
• mild disease ( erythema multiforme minor)

• outpatient treatment with topical steroids
• dermatology follow-up

• severe disease ( Stevens-Johnson syndrome)

• a short course of high intensity steroid treatment may be of benefit
• intravenous immune globulin controversial [2]
• treat secondary infections
• no role for prophylactic antibiotics [2]

• ophthalmology consult for eye involvement
• prognosis: mortality 10% generally due to infection

More General Terms

• skin disease (dermatologic disorder, dermatopathy, dermatosis)
• syndrome

Additional Terms

• erythema multiforme
• pharmaceutical agents causing severe cutaneous adverse reactions (SCARs)
• toxic (bullous) epidermal necrolysis (Lyell syndrome, TEN)

References

1. H. Quinny Cheng, UCSF Fresno lecture, Oct 21, 1998
2. Medical Knowledge Self Assessment Program (MKSAP) 15, American College of Physicians, Philadelphia 2009
3. Carr DR et al Approach to the acute, generalized, blistering patient. Semin Cutan Med Surg. 2007 Sep;26(3):139-46. Review. PMID: [1]
4. Cotliar J. Approach to the patient with a suspected drug eruption. Semin Cutan Med Surg. 2007 Sep;26(3):147-54. Review. PMID: [2]

Stevens-Johnson syndrome (SJS)