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QuestionChoice
Stevens-Johnson syndrome typically produces a maculopapular rash with target lesions. There is normally mucosal involvement, as can be seen in the image above. Stevens-Johnson syndrome is usually drug-induced. This will require a hospital admission for treatment
31808
a
Stevens-Johnson syndrome
QuestionChoice
SCC would not establish over such a short time frame
31811
d
Squamous cell carcinoma (SCC) of the skin
QuestionChoice
Pyoderma gangrenosum usually manifests as a painful pustule or nodule with rapid progression. There is normally just one nodule, and is usually on the lower limbs. There is no mucosal involvement. It can sometimes be accompanied by fever and systemic symptoms
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c
Pyoderma gangrenosum
QuestionChoice
This is an autoimmune condition causing blistering urticarial lesions. This can sometimes have mucosal involvement. It is not drug induced, as this question is implying is the cause of this man's rash. It does not normally produce systemic symptoms
31809
b
Bullous Pemphigoid
QuestionChoice
Eczema herpeticum is a disseminated infection of a herpes virus that produces itchy blisters or punched-out erosions. It is normally seen in children with eczema. It can be accompanied by systemic symptoms such as fever
31812
e
Eczema herpeticum
Concept
Chapter
# Summary

Stevens-Johnson syndrome (SJS) is a serious, immune-complex-mediated hypersensitivity disorder often triggered by medication or, less commonly, viral, bacterial, or fungal infections. Clinically, it's characterized by symptoms resembling an upper respiratory tract infection, followed by erythematous macules and mucosal ulceration, affecting less than 10% of the body surface. Diagnosis is usually clinical, supplemented by skin biopsy. Management is largely supportive, with a focus on skin and eye care.

# Definition

Stevens-Johnson syndrome (SJS) is an immune-complex-mediated hypersensitivity disorder. It ranges from mild to severe forms, part of a spectrum that includes toxic epidermal necrolysis (TEN) at its most severe end.

# Epidemiology

SJS is a rare disorder, with an incidence estimated between 2.6 to 6.1 cases per million people annually. It affects both genders and all age groups, although some studies suggest a slightly higher incidence in females and middle-aged adults.

# Pathophysiology

The predominant cause of SJS is adverse drug reactions, most commonly from sulfonamides, beta-lactams (penicillins and cephalosporins), antiepileptics, allopurinol, and NSAIDs. Infectious agents, particularly viral pathogens like herpes simplex virus, Epstein-Barr virus, HIV, influenza, and hepatitis, can also trigger SJS. Bacterial and fungal infections are much less common causes.

# Signs and Symptoms

- SJS often presents within a week of medication intake, initially resembling an upper respiratory tract infection with symptoms such as cough, cold, fever, and sore throat.
- Erythematous macules, later becoming target-shaped, appear after a few days.
- Flaccid blisters develop and the Nikolsky sign is positive.
- SJS affects less than 10% of the body surface, in contrast to TEN, which involves more than 30% of the skin.
- Mucosal ulceration is seen in at least two of the following: conjunctiva, mouth, urethra, pharynx, or gastrointestinal tract. 

SJS has a 10% mortality rate, primarily due to dehydration, infection, or disseminated intravascular coagulation. In comparison, TEN has a 30% mortality rate.

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# Differential Diagnosis

The differential diagnosis of SJS includes:

- Erythema Multiforme: Characterized by target lesions, typically on the hands and feet, and less severe mucosal involvement.
- Drug Rash with Eosinophilia and Systemic Symptoms (DRESS): Presents with fever, rash, and internal organ involvement, often with a delay of 2-6 weeks after drug exposure.
- Acute Generalized Exanthematous Pustulosis (AGEP): Noted for the rapid development of numerous small non-follicular sterile pustules on a background of edematous erythema.

# Investigations

The diagnosis of SJS is primarily clinical but can be supported by a skin biopsy, which can reveal necrotic keratinocytes and a sparse lymphocytic infiltrate.

# Management

Management of SJS is largely supportive, focusing on skin care and prevention of ocular complications through timely ophthalmology referrals. Patients may require hospitalization for fluid and electrolyte management, pain control, and potential treatment of secondary infections.

# References

[Click here for more information on Stevens-Johnson syndrome](https://patient.info/doctor/stevens-johnson-syndrome)
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lecFHAgnNocIyIxsWKgIdINAOA==
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Dermatology
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Stevens-Johnson syndrome 
Topic
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Dermatology
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Stevens-Johnson syndrome is a severe drug-induced condition characterised by a maculopapular rash, often with mucosal involvement, requiring urgent medical attention.
Picture
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Stevens-johnson-syndrome.jpeg
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Dermatology
A 40-year-old man presents to the accident and emergency department with a painful rash which has established quickly over the last few hours. He is pyrexial and reports general arthralgia. He was started on a course of amoxicillin for sinusitis the preceding day. He has no significant past medical or dermatological history.

His rash can be seen below.

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Which of the following is the most likely diagnosis in this patient?
a