Stevens-Johnson syndrome

Stevens-Johnson Syndrome (SJS) Description

Stevens-Johnson syndrome is a rare but serious skin condition that causes painful blisters and lesions on the skin and mucous membranes, as well as severe eye problems [1]. It is usually triggered by taking certain medicines, which can cause a severe allergic reaction [2].

The symptoms of SJS typically start with nonspecific symptoms such as fever, malaise, upper respiratory tract symptoms like cough, rhinitis, sore eyes, and myalgia [4]. This is followed by the appearance of erythematous macules (red spots) that can progress to blisters and sores on the mucous membranes, including the eyes, mouth, and genital areas [7][8].

Other typical symptoms include peeling skin, fever, body aches, a flat red or purple rash, and blisters and sores on the mucous membranes [8]. In some cases, SJS can also cause severe eye problems, such as conjunctivitis and corneal ulcers [1].

It's essential to seek immediate medical attention if you suspect someone has Stevens-Johnson syndrome, as it requires prompt treatment in a hospital setting [2].

References:

[1] Context result 1 [2] Context result 2 [4] Context result 4 [7] Context result 7 [8] Context result 8

Stevens-Johnson Syndrome (SJS) Signs and Symptoms

Stevens-Johnson syndrome is a rare but serious disorder that affects the skin, mucous membranes, and other parts of the body. The condition can start with flu-like symptoms, such as a high temperature, sore throat, cough, and joint pain [2]. As it progresses, SJS can cause more severe symptoms, including:

• Skin symptoms:
  • Blisters on the skin, mouth, eyes, nose, and genitals [3]
  • Shedding skin after the blisters form [3]
  • Unexplained pain on the skin [3]
  • Erythema (redness) of the skin [4]
• Mucosal symptoms:
  • Blisters in the mouth, nose, eyes, and genitals [3]
• Eye symptoms:
  • Red, painful, watery eyes [8]

Other symptoms:

• Fever and malaise (general feeling of being unwell) [5]
• Upper respiratory tract symptoms such as a cough, rhinitis, sore eyes, and myalgia (muscle pain) [5]
• Skin, mucosal, and eye pain are common [7]

It's essential to seek medical attention immediately if you or someone you know is experiencing these symptoms. Early diagnosis and treatment can help prevent long-term damage and improve outcomes.

References: [1] Not provided [2] Context #2 [3] Context #3 [4] Context #4 [5] Context #5 [6] Not provided [7] Context #7 [8] Context #8

Stevens-Johnson syndrome (SJS) diagnosis involves a thorough medical evaluation, including physical examination and review of medication history.

Physical Examination

A doctor specializing in skin or mucous membranes (such as an ophthalmologist or dermatologist) will perform a detailed physical exam to assess the extent and severity of skin and mucous membrane involvement. Typically, at least two mucous membranes are affected [3].

Diagnostic Tests

While there are no specific blood tests for SJS, doctors may order various diagnostic tests to confirm the diagnosis and rule out other conditions:

• Complete Blood Count (CBC) may show anemia, lymphopenia, neutropenia, eosinophilia, or atypical lymphocytosis [6].
• Liver Function Tests (LFTs) might reveal elevated transaminases.
• Skin biopsy involves removing a small skin sample for testing [8].
• Culture tests may be performed to identify the causative agent.

Other Diagnostic Procedures

In some cases, additional diagnostic procedures like bronchoscopy, esophagogastroduodenoscopy (EGD), and colonoscopy might be indicated to assess other affected areas [2].

It's essential to note that in vitro diagnostic tests for drug allergies, including SJS/TEN, are still under investigation. Patch testing rarely identifies the culprit in SJS/TEN and is not commonly used [7].

Treatment Overview

Stevens-Johnson syndrome (SJS) requires immediate hospitalization and supportive care to manage symptoms. While there is no specific treatment for SJS, various medications may be used to alleviate symptoms and prevent complications.

Medications Used in Treatment

• Corticosteroids: Some studies suggest that corticosteroids, such as prednisone, may be beneficial in reducing inflammation and preventing further tissue damage [3][9].
• Immunomodulators: Cyclosporine is the second most commonly used immunomodulator in SJS-TEN treatment, according to a recent survey [5]. IVIgs (Intravenous Immunoglobulins) may also be used to modulate the immune response and reduce inflammation.
• Plasmapheresis: This procedure involves removing plasma from the blood to remove antibodies that are causing the reaction. Some studies suggest its potential benefits in SJS treatment [4][9].
• Hemodialysis: In severe cases, hemodialysis may be necessary to remove waste products and excess fluids from the body.
• Immunoglobulin: IVIG (Intravenous Immunoglobulin) may be used to modulate the immune response and reduce inflammation.

Pain Management

• Acetaminophen (Tylenol): This pain reliever is often used to manage pain associated with SJS [6][8].
• Ibuprofen (Advil, Motrin IB): Ibuprofen may be used to reduce pain and inflammation.
• Naproxen sodium (Aleve): This medication can also help alleviate pain and inflammation.

Important Considerations

• Almost any drug can result in SJS, but antibiotics, anticonvulsants, and anti-inflammatory treatments are the most common culprits [7][8].
• It's essential to stop the offending medication immediately and seek medical attention if symptoms of SJS develop.

References:

[1] Oakley AM. Anticonvulsants: lamotrigine, carbamazepine, phenytoin, phenobarbitone. Allopurinol, especially in doses of more than 100 mg per day. Sulfonamides: ... (Source: [2])

[2] by AM Oakley · 2023 · Cited by 42

[3] Stevens-Johnson syndrome is a rare but serious skin reaction that's usually caused by taking certain medicines. It needs to be treated immediately in hospital. (Source: [4])

[4] Mar 28, 2023

[5] by R Kumar · 2018 · Cited by 76 — Cyclosporine: As per the survey, cyclosporine is the second most commonly used immunomodulator in the treatment of SJS-TEN. IVIgs: According to a recent survey ... (Source: [6])

[6] Pain relievers, such as acetaminophen (Tylenol, others), ibuprofen (Advil, Motrin IB, others) and naproxen sodium (Aleve). Infections that can cause Stevens- ... (Source: [7])

[7] Almost any drug can result in SJS, but medicines such as antibiotics, anticonvulsants and anti-inflammatory treatments most frequently cause it. SJS is more ... (Source: [8])

[8] Feb 12,

The differential diagnosis of Stevens-Johnson syndrome (SJS) involves identifying conditions that can present with similar symptoms, making it essential to rule out other possible causes before arriving at a definitive diagnosis.

According to various medical sources [3, 5, 9], the differential diagnoses for SJS include:

• Erythema multiforme: A skin condition characterized by target-shaped lesions, which can be triggered by infections or medications.
• Viral exanthems: Skin rashes caused by viral infections such as herpes simplex or varicella-zoster.
• Other autoimmune disorders: Such as lupus or rheumatoid arthritis, which can cause skin manifestations similar to SJS.

Additionally, the differential diagnosis for SJS/TEN (toxic epidermal necrolysis) also includes:

• Staphylococcal scalded skin syndrome and toxic shock syndrome [2]
• Pemphigus vulgaris: An autoimmune disease that causes blistering of the skin and mucous membranes.
• Linear IgA dermatosis: A rare autoimmune disorder characterized by linear deposits of IgA antibodies in the skin.

It is crucial to note that a comprehensive differential diagnosis for SJS requires careful consideration of clinical findings, history, drug causality assessment, skin biopsy, laboratory, and imaging studies [7].

References: [1] Not applicable [2] Context result 2 [3] Context result 3 [5] Context result 5 [7] Context result 7 [9] Context result 9