Stevens-Johnson Syndrome Questions & Answers

Updated: Jan 17, 2019
  • Author: C Stephen Foster, MD, FACS, FACR, FAAO, FARVO; Chief Editor: Andrew A Dahl, MD, FACS  more...
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Questions & Answers

Overview

What is Stevens-Johnson syndrome (SJS) and what are the classifications of severity?

What are the prodromal symptoms of Stevens-Johnson syndrome (SJS)?

What are the symptoms of Stevens-Johnson syndrome (SJS) and how are cutaneous lesions characterized?

What are the signs of mucosal involvement in Stevens-Johnson syndrome (SJS)?

What ocular signs may be noted on slit-lamp exams in Stevens-Johnson syndrome (SJS)?

What are typical histopathologic findings in skin specimens in the workup of Stevens-Johnson syndrome (SJS)?

What are the ocular exam findings in Stevens-Johnson syndrome (SJS)?

How is Stevens-Johnson syndrome (SJS) treated?

What is the recommended treatment of nonviable epidermis in Stevens-Johnson syndrome (SJS)?

What is the initial treatment of acute ocular manifestations in Stevens-Johnson syndrome (SJS)?

How are mild chronic superficial keratopathy and severe ocular involvement treated in Stevens-Johnson syndrome (SJS)?

What is Stevens-Johnson syndrome (SJS) and how does the presentation vary?

When and how was Stevens-Johnson syndrome (SJS) first described, and what is the background of Stevens-Johnson syndrome?

What are the types of Stevens-Johnson syndrome (SJS) and why is the classification scheme the subject of debate?

What is the etiology of Stevens-Johnson syndrome (SJS)?

Which procedure is needed to establish a definitive diagnosis of Stevens-Johnson syndrome (SJS) and how is it treated?

Where can patient education information on Stevens-Johnson syndrome (SJS) be found?

What is the pathogenesis of Stevens-Johnson syndrome (SJS) and which patient populations are at greatest risk?

What is a slow acetylator and what is the role of acetylation in the pathophysiology of Stevens-Johnson syndrome (SJS)?

What is the role of cytokines in the pathophysiology of Stevens-Johnson syndrome (SJS)?

What is the role of allopurinol and lamotrigine in the pathogenesis of Stevens-Johnson syndrome (SJS) and how is the risk mitigated?

What are the etiologic categories of Stevens-Johnson syndrome (SJS)?

How common is idiopathic Stevens-Johnson syndrome (SJS) and how do the etiologies differ in adults and children?

Which viral diseases have been associated with Stevens-Johnson syndrome (SJS)?

Which viruses are associated with the development of Stevens-Johnson syndrome (SJS) in children?

What are the bacterial causes of Stevens-Johnson syndrome (SJS)?

What are the fungal and protozoal causes of Stevens-Johnson syndrome (SJS)?

What are the most common drug-related causes of Stevens-Johnson syndrome (SJS)?

Which anticonvulsants may cause Stevens-Johnson syndrome (SJS)?

Which antiretroviral drugs may cause Stevens-Johnson syndrome (SJS)?

Which drugs are less commonly implicated as a cause of Stevens-Johnson syndrome (SJS)?

Which human leukocyte antigens are associated with increased risk for Stevens-Johnson syndrome (SJS)?

What are considerations regarding genetic factors in the etiology of Stevens-Johnson syndrome (SJS)?

What is the epidemiology of Stevens-Johnson syndrome (SJS)?

Does Stevens-Johnson syndrome (SJS) have a racial predilection?

Is Stevens-Johnson syndrome (SJS) more common in men or women?

What are the age-related demographics of Stevens-Johnson syndrome (SJS)?

What is the prognosis of Stevens-Johnson syndrome (SJS)?

What is the mortality rate for Stevens-Johnson syndrome (SJS) and which complications are associated with increased mortality?

How is the risk for death calculated for Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN)?

What are the secondary negative prognostic factors in Stevens-Johnson syndrome (SJS)?

How is the severity of the cutaneous reaction a risk factor for mortality in Stevens-Johnson syndrome (SJS)?

What is the prognosis of Stevens-Johnson syndrome (SJS)?

What long-term complications are associated with Stevens-Johnson syndrome (SJS)?

Presentation

How is the initial presentation of Stevens-Johnson syndrome (SJS) characterized?

What does a history of fever or localized worsening suggest in Stevens-Johnson (SJS) syndrome?

How are oral and/or mucous membranes and genitourinary involvement characterized in Stevens-Johnson syndrome (SJS)?

What causes recurrences of Stevens-Johnson syndrome (SJS)?

What are the prodromal symptoms of Stevens-Johnson syndrome (SJS)?

How is the rash characterized in the initial presentation of Stevens-Johnson syndrome (SJS)?

Where on the body are lesions found in Stevens-Johnson syndrome (SJS)?

Which ocular symptoms are associated with Stevens-Johnson syndrome (SJS)?

What is the role of drug exposure in the history for Stevens-Johnson syndrome (SJS)?

How are lesions characterized in the physical exam in Stevens-Johnson syndrome (SJS)?

Which body parts are most commonly affected by lesions in Stevens-Johnson syndrome (SJS)?

How is mucosal involvement characterized in Stevens-Johnson syndrome (SJS)?

Can a diagnosis of Stevens-Johnson syndrome (SJS) be established in the absence of skin lesions?

Which physical signs may be noted on exam in Stevens-Johnson syndrome (SJS)?

Which ocular signs may be noted on slit-lamp exam in Stevens-Johnson syndrome (SJS)?

How common is severe ocular disease in Stevens-Johnson syndrome (SJS) and which ocular complications occur?

What are the nonocular complications of Stevens-Johnson syndrome (SJS)?

How long can lesions erupt in Stevens-Johnson syndrome (SJS) and what mucosal complications may occur?

Which ocular, genitourinary, and cutaneous complications occur in Stevens-Johnson syndrome (SJS)?

DDX

Which condition should be considered in all patients presenting with target lesions and mucous membrane involvement?

What other conditions should be considered in the differential diagnosis of Stevens-Johnson syndrome (SJS)?

What are the differential diagnoses for Stevens-Johnson Syndrome?

Workup

Which procedure is needed for a definitive diagnosis of Stevens-Johnson syndrome (SJS)?

Which serum levels are typically elevated in Stevens-Johnson syndrome (SJS)?

What does the WBC count indicate in Stevens-Johnson syndrome (SJS) and what other testing may be needed to help manage related problems?

How are skin and blood cultures used in the workup of Stevens-Johnson syndrome (SJS)?

Which skin biopsy findings are associated with Stevens-Johnson syndrome (SJS)?

Which procedures and imaging studies are indicated in the workup of Stevens-Johnson syndrome (SJS)?

How are typical histopathologic findings characterized in Stevens-Johnson syndrome (SJS)?

What are dermal and epidermal histopathologic findings in Stevens-Johnson syndrome (SJS)?

What is the role of CD8+ T cells in Stevens-Johnson syndrome (SJS)?

What are the histologic findings in conjunctival biopsies of active ocular disease in Stevens-Johnson syndrome (SJS)?

What does immunohistology of the conjunctiva demonstrate in Stevens-Johnson syndrome (SJS)?

How are conjunctival histologic findings characterized on transmission electron microscopy in Stevens-Johnson syndrome (SJS)?

What is the role of vivo confocal microscopy in the workup of Stevens-Johnson syndrome (SJS)?

Treatment

How is Stevens-Johnson syndrome (SJS) treated?

What approach should paramedics take in the treatment of Stevens-Johnson syndrome (SJS)?

What is the role of emergency department (ED) care in the treatment of Stevens-Johnson syndrome (SJS)?

How are oral lesions managed in Stevens-Johnson syndrome (SJS)?

How are skin lesions treated in Stevens-Johnson syndrome (SJS)?

How is fluid management provided in the treatment of Stevens-Johnson syndrome (SJS) and how much fluid is needed?

What is the supportive systemic therapy after the second day of hospitalization in Stevens-Johnson syndrome (SJS)?

What pulmonary care is indicated in the treatment of Stevens-Johnson syndrome (SJS)?

How is the risk of nosocomial infection reduced in Stevens-Johnson syndrome (SJS)?

Why should silver sulfadiazine be avoided in the treatment of Stevens-Johnson syndrome (SJS), and which antiseptic should be used instead?

When are prophylactic systemic antibiotics and antimicrobials used for infection control in Stevens-Johnson syndrome (SJS)?

What are the first signs of sepsis in Stevens-Johnson syndrome (SJS) and what is the treatment?

How is an antibiotic selected for infection control in Stevens-Johnson syndrome (SJS)?

What are the recommended treatments for skin care in Stevens-Johnson syndrome (SJS) and which biologic dressings are used?

What is the criterion standard in the treatment of Stevens-Johnson syndrome (SJS)?

Why are randomized studies with a placebo control lacking in Stevens-Johnson syndrome (SJS)?

Which therapeutic modalities have been advocated in the treatment of Stevens-Johnson syndrome (SJS)?

What is the role of systemic steroids in the treatment of Stevens-Johnson syndrome (SJS)?

What is the role of cyclosporine in the treatment of Stevens-Johnson syndrome (SJS)?

What is the role of IVIG in the treatment of Stevens-Johnson syndrome?

What is the initial treatment of acute ocular manifestations in Stevens-Johnson syndrome (SJS)?

How is ocular integrity preserved in Stevens-Johnson syndrome (SJS)?

When is visual rehabilitation indicated in Stevens-Johnson syndrome (SJS)?

What is the treatment for mild chronic superficial keratopathy in Stevens-Johnson syndrome (SJS)?

How is visual rehabilitation of severe ocular involvement characterized in Stevens-Johnson syndrome (SJS)?

How are chronic ocular manifestations of Stevens-Johnson syndrome (SJS) treated?

How is corneal clarity preserved after visual reconstruction for chronic ocular manifestations of Stevens-Johnson syndrome (SJS)?

What is involved in the long-term management of chronic ocular manifestations of Stevens-Johnson syndrome (SJS)?

What consultations should be considered for patients with Stevens-Johnson syndrome (SJS)?

What is the long-term management of Stevens-Johnson syndrome (SJS)?

Guidelines

What are the treatment guidelines for eye involvement in Stevens-Johnson syndrome (SJS)?

Medications

What is the goal of pharmacotherapy in Stevens-Johnson syndrome (SJS)?

When is the use of antibiotics mandated in Stevens-Johnson syndrome (SJS) and which organisms are commonly involved in bloodstream infection?

What is the role of systemic corticosteroids in the treatment of Stevens-Johnson syndrome (SJS)?

What is the role of human IVIG in the treatment of Stevens-Johnson syndrome (SJS)?

Which medications in the drug class Immune Globulins are used in the treatment of Stevens-Johnson Syndrome?

Which medications in the drug class Immunosuppressants are used in the treatment of Stevens-Johnson Syndrome?

Which medications in the drug class Corticosteroids are used in the treatment of Stevens-Johnson Syndrome?