Stevens-Johnson Syndrome

Stevens-Johnson Syndrome
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Synonyms
Pronounce
Specialty	Dermatology, Immunology
Symptoms	Severe rash, skin peeling, sores in mucous membranes
Complications	Sepsis, blindness, pneumonia
Onset
Duration
Types
Causes	Drug reactions, infections
Risks	Genetic factors, certain medications
Diagnosis	Clinical examination, biopsy
Differential diagnosis	Toxic epidermal necrolysis, Erythema multiforme
Prevention	Avoidance of known triggers
Treatment	Hospitalization, stopping offending drugs, supportive care
Medication	Corticosteroids, Immunoglobulins
Prognosis	Variable
Frequency	Rare
Deaths

Stevens-Johnson Syndrome (SJS) is a rare, serious disorder of the skin and mucous membranes. It is usually a reaction to a medication or an infection. Often, it begins with flu-like symptoms, followed by a painful red or purplish rash that spreads and blisters, eventually causing the top layer of the skin to die and shed.

Symptoms and Signs

The primary symptoms of SJS include:

• Fever
• Unexplained widespread skin pain
• A red or purple skin rash that spreads
• Blisters on the skin and the mucous membranes of the mouth, nose, eyes, and genitals
• Shedding of the skin

Causes

SJS is typically caused by an adverse reaction to medications, including antibiotics, anticonvulsants, and NSAIDs. It can also be triggered by infections such as herpes simplex virus or HIV.

Pathophysiology

The exact mechanism of Stevens-Johnson Syndrome is not well understood, but it is believed to involve a dysfunction of the immune system. The immune response results in significant damage to the epithelial cells of the skin and mucous membranes.

Diagnosis

Diagnosis of SJS is primarily based on the clinical presentation of the symptoms. A biopsy of the affected skin can be performed to confirm the diagnosis. It is important to distinguish SJS from other similar conditions such as toxic epidermal necrolysis (TEN) and erythema multiforme.

Treatment

Treatment of SJS typically requires hospitalization. The first step is to discontinue any medications that might have triggered the syndrome. Treatment focuses on symptom relief, wound care, and preventing complications. This may include:

• Pain management
• Rehydration and nutritional support
• Topical treatment for skin and mucous membrane lesions
• Use of corticosteroids and immunoglobulins has been controversial and is decided on a case-by-case basis.

Prognosis

The prognosis for SJS can vary. With prompt treatment, most individuals with SJS can recover within several weeks. However, the condition can be life-threatening and may result in permanent skin damage and other serious complications.

Epidemiology

SJS is a rare condition, affecting 1-2 per million people annually. It can occur in any age group but is more common in older adults. Certain genetic predispositions can increase the risk of developing SJS.

See also

• Toxic epidermal necrolysis
• Erythema multiforme
• Drug reaction with eosinophilia and systemic symptoms (DRESS)