Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN):

Question 1

Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN):

SJS Definition:

Answer 1

• SJS is the less severe condition, in which skin detachment is <10% of the body surface
• TEN involves detachment of >30% of the body surface area (BSA)

Question 2

Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN):

SJS/TEN overlap:

Answer 2

• SJS/TEN overlap describes patients with skin detachment of 10 to 30% of BSA
• We will use the term “SJS/TEN” to refer collectively to SJS, TEN, and SJS/TEN overlap syndrome

Question 3

Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN):

What are SJS and TEN?

Answer 3

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe mucocutaneous reactions, most commonly triggered by medications, characterized by extensive necrosis and detachment of the epidermis

Question 4

Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN):

Mostly affected sites:

Answer 4

Mucous membranes are affected in over 90% of patients, usually at two or more distinct sites (ocular, oral, and genital)

Question 5

Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN):

Risk Factors SJS/TEN:

Answer 5

1. High doses of medications
2. HIV
3. Genetic factors
4. Malignacy
5. Physical stimuli
6. SLE

Question 6

Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN):

Clinical Presentation

Answer 6

• Prodrome
• Fever, often exceeding 39°C and influenza-like symptoms precede by one to three days the development of mucocutaneous lesions Photophobia, conjunctival itching or burning, and pain on swallowing may be early symptoms of mucosal involvement
• Malaise, myalgia, and arthralgia are present in most patients

Question 7

Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN):

Signs and symptoms that should alert the clinician to the possibility of SJS/TEN include:

Answer 7

• In some patients, an exanthematous eruption can be the heralding sign of SJS/TEN

Signs and symptoms that should alert the clinician to the possibility of SJS/TEN include:

1. fever >38°C
2. mucositis
3. skin tenderness
4. blistering

Question 8

Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN):

Cutaneous lesions:

Answer 8

• The skin lesions typically begin with ill-defined, coalescing, erythematous macules with purpuric centers, although many cases of SJS/TEN may present with diffuse erythema
• The skin is often tender to the touch, and skin pain can be prominent and out of proportion to the cutaneous findings
• Lesions start on the face and thorax before spreading to other areas and are symmetrically distributed
• The scalp is typically spared, and palms and soles are rarely involved Atypical target lesions with darker centers may be present As the disease progresses, vesicles and bullae form, and within days the skin begins to slough

Question 9

Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN):

Mucosal lesions:

Answer 9

Mucosal involvement occurs in approximately 90% of cases of SJS/TEN and can precede or follow the skin eruption. Painful crusts and erosions may occur on any mucosal surface

Question 10

Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN):

Oral involvement:

Answer 10

The oral mucosa and the vermilion border are almost invariably involved, with painful hemorrhagic erosions covered with a grayish- white membrane Stomatitis and mucositis lead to impaired oral intake with consequent malnutrition and dehydration

Question 11

Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN):

Ocular involvement:

Answer 11

• Ocular — Ocular involvement is reported in approximately 80% of patients. The most common change in the eyes is a severe conjunctivitis with a purulent discharge, but bullae may develop
• Corneal ulceration is frequent, and anterior uveitis or panophthalmitis may occur. Pain and photophobia are accompanying symptoms

Question 12

Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN):

Management:

Answer 12

The main principles of supportive care are the same as for major burns and include wound care, fluid and electrolyte management, nutritional support, temperature management, pain control, and monitoring or treatment of superinfections

1. Systemic corticosteroids — The use of systemic corticosteroids in patients remains controversialIntravenous immune globulin — Data are limited and conflicting
2. Intravenous immune globulin plus systemic corticosteroids — the data are too limited to draw any firm conclusions
3. Cyclosporine — cyclosporine given at the dose of 3 to 5 mg/kg per day may slow the progression of SJS/TEN, in the absence of significant toxicity
4. Tumor necrosis factor inhibitors — A single infusion of 5 mg/kg of the TNF- alpha inhibitor infliximab halted the progression of skin detachment and induced a rapid re-epithelization of the denuded skin