STEVENS-JOHNSON SYNDROME

Stevens-Johnson Syndrome (SJS) is a rare but serious disorder characterized by a severe and potentially life-threatening skin reaction. It is considered a medical emergency and requires prompt diagnosis and treatment. SJS is part of a spectrum of conditions known as severe cutaneous adverse reactions (SCARs), which also include toxic epidermal necrolysis (TEN). Understanding the causes, symptoms, diagnosis, treatment, and prognosis of Stevens-Johnson Syndrome is crucial for effective management and prevention of complications.

SYMPTOMS

The hallmark symptom of Stevens-Johnson Syndrome is the sudden onset of a widespread, painful rash that rapidly progresses over hours to days. Other common symptoms may include:

  • Fever: High fever may accompany the onset of the rash, indicating systemic inflammation and illness.
  • Flu-like Symptoms: Malaise, fatigue, headache, and muscle aches may precede or accompany the rash.
  • Mucous Membrane Involvement: In addition to skin involvement, SJS can affect mucous membranes, leading to painful lesions in the mouth, throat, eyes, genitals, and gastrointestinal tract.
  • Blisters and Skin Detachment: The rash may progress to form blisters, erosions, and widespread skin detachment, resembling a severe burn or scald injury.

The severity of symptoms varies depending on the extent of skin and mucous membrane involvement, with SJS-TEN overlap syndrome representing an intermediate form of disease severity.

DIAGNOSIS

Diagnosing Stevens-Johnson Syndrome typically involves a combination of clinical evaluation, medical history, and laboratory tests. Key diagnostic considerations include:

  • Clinical Presentation: Characteristic features of SJS include the sudden onset of a widespread, painful rash with mucous membrane involvement.
  • Medical History: Inquiring about recent medication use, infections, vaccinations, or systemic illnesses can provide valuable clues to the underlying cause.
  • Skin Biopsy: A skin biopsy may be performed to confirm the diagnosis and rule out other skin conditions with similar features.
  • Laboratory Tests: Blood tests such as complete blood count (CBC), liver function tests, and tests for infectious agents (e.g., herpes simplex virus, Mycoplasma pneumoniae) may be performed to assess for underlying triggers or complications.

Prompt referral to a dermatologist or specialist in severe cutaneous adverse reactions is essential for accurate diagnosis and timely intervention.

TREATMENT

Treatment of Stevens-Johnson Syndrome is primarily supportive and aims to address symptoms, prevent complications, and promote healing. Key components of treatment may include:

  • Hospitalization: Patients with SJS require hospitalization in a specialized burn unit or intensive care unit (ICU) for close monitoring and supportive care.
  • Fluid and Electrolyte Management: Intravenous fluids and electrolyte replacement may be administered to maintain hydration and electrolyte balance.
  • Pain Management: Analgesic medications such as acetaminophen or opioids may be prescribed to relieve pain and discomfort.
  • Wound Care: Gentle cleansing and topical wound care measures may be used to promote healing and prevent infection.
  • Systemic Medications: Systemic corticosteroids or immunomodulatory agents may be considered in certain cases to suppress inflammation and modulate the immune response.

Treatment decisions are individualized based on the severity of symptoms, extent of skin involvement, underlying triggers, and presence of complications.

Related Articles

JUVENILE IDIOPATHIC ARTHRITIS

Overview and FactsTypes and SymptomsDiagnosis & MedicationsOverview and Facts Juvenile idiopathic arthritis (JIA), formerly known as juvenile rheumatoid arthritis, is [...]

JUVENILE POLYPOSIS SYNDROME

Overview and FactsTypes and SymptomsDiagnosis & MedicationsOverview and Facts Juvenile polyposis syndrome (JPS) is a rare genetic disorder characterized by [...]

JUVENILE PILOCYTIC ASTROCYTOMA

Overview and FactsTypes and SymptomsDiagnosis & MedicationsOverview and Facts Juvenile pilocytic astrocytoma (JPA) is a relatively common type of brain [...]