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A 42-year-old woman comes to the emergency department with painful skin lesions. She
is unable to eat. or drink due to painful blistering and ulceration in her mouth and throat.
The rash was preceded by fever, headache, sore throat, cough, nausea, and vomiting.
She was treated for acute cystitis with trimethoprim-sulfamethoxazole 5 days ago, but her
past history is otherwise unremarkable. Her temperature is 38.3 C (101 F), blood
pressure is 95/60 mm Hg, pulse is 110/min, and respirations are 14/min. On examination,
the skin is hot and tender with widespread confluent erythematous macules that cover
most of the visible skin surface. The oral mucosa shows blistering and erosions. A
photograph of her back is shown below..

What is the most likely diagnosis?

0 A Erythema multiforme
0 B. Exfoliative dermatitis
0 C. Staphylococcal scalded skin syndrome
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She was treated for acute cystitis with trimethoprim-sulfamethoxazole 5 days ago, but her
past history is otherwise unremarkable. Her temperature is 38:3 C (101 F), blood
pressure is 95/60 mm Hg, pulse is 110/min, and respirations are 14/min. On examination,
the skin is hot and tender with widespread confluent erythematous macules that cover
most of the visible skin surface. The oral mucosa shows blistering and erosions. A
photograph of her back is shown below.

What is the most likely diagnosis?

0 A Erythema multiforme
0 B. Exfoliative dermatitis
0 C. Staphylococcal scalded skin syndrome
0 D. Stevens-Johnson syndrome
0 E. Toxic epidermal necrolysis

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A 42-year-old woman comes to the emergency department with painful skin lesions. She
is unable to eat or drink due to painful blistering and ulceration in her mouth and throat.
The rash was preceded by fever, headache, sore throat, cough, nausea, and vomiting.
She was treated for acute cystitis with trimethoprim-sulfamethoxazole 5 days ago, but her
past history is otherwise unremarkable. Her temperature is 38.3 C (101 F), blood
pressure is 95/60 mm Hg, pulse is 11 0/min, and respirations are 14/min. On examination,
the skin is hot and tender with widespread confluent erythematous macules that cover
most of the visible skin surface. The oral mucosa shows blistering and erosions. A
photograph of her back is shown below.

What is the most likely diagnosis?

A. Erythema multiforme [0%)

8. Exfoliative dermatitis [0%)
C. Staphylococcal scalded skin syndrome [4%)
 What is the most likely diagnosis?

A. Erythema multiforme [0%)

8. Exfoliative dermatitis [0%)
C . Staphylococcal scalded skin syndrome [4%)
D. Stevens-Johnson syndrome [26%)
E. Toxic epidermal necrolysis [69%)

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Explanation: User ld

Stevens-Johnson syndrome & toxic

epidermal necrolysis

<10% of BSA: SJS

Nomenclature 10%-30%: SJS!TEN overlap
>30%: TEN

• 4-28 days after exposure to trigger

(2 days after repeat exposure)
• Acute influenza-like prodrome
Clinical
• Rapid-onset erythematous macules,
features
vesicles, bullae.
• Necrosis & sloughing of epidermis
• Mucosal involvement

Drugs
• Allopurinol
• Antibiotics (eg, sulfonamides)
• Anticonvulsants (eg, carbamazepine,
lamotrigine. phenytoin)
Common • NSAIDs (eg, piroxicam)
triggers
• Sulfasalazine
Other

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Stevens-Johnson syndrome & toxic

epidermal necrolysis

<10% of BSA: SJS

Nomenclature 10%-30%: SJS!TEN overlap
>30%: TEN

• 4-28 days after exposure to trigger

(2 days after repeat exposure)
• Acute influenza-like prodrome
Clinical
• Rapid-onset erythematous macules,
features
vesicles, bullae.
• Necrosis & sloughing of epidermis
• Mucosal involvement

Drugs
• Allopurinol
• Antibiotics (eg, sulfonamides)
• Anticonvulsants (eg, carbamazepine,
lamotrigine. phenytoin)
Common • NSAIDs (eg, piroxicam)
t riggers
• Sulfasalazine
Other
• Mycoplasma pneumoniae
• Vaccination
• Graft-vs-host disease

BSA = body surface area; NSAIOs = nonsteroidal anti-inflammatory drugs;

SJS = Stevens-Johnson syndrome; TEN= toxic epidermal necrolysis.
© UWortd

This patient's clinical features - acute mucocutaneous disorder with associated systemic
toxicity, following use of sulfonamide antibiotics - are consistent with toxic epidermal
necrolysis (TEN). TEN is an inflammatory hypersensitivity reaction to drugs (especially
sulfonamides, nonsteroidal antiinflammatory drugs, anticonvulsants and allopurinol) or
certain infections (usually Mycoplasma pneumoniae). Clinical features of TEN are
variable, but typically include coalescing erythematous macules, bullae, desquamation,
and mucositis. Patches of skin slide off with light pressure (positive Nikolsky's
sign). Systemic signs are common and may include fever, tachycardia, hypotension,
altered level of consciousness, conjunctivitis, seizures, and coma. The exact

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BSA = body surface area; NSAIDs = nonsteroidal anti-inflammatory drugs;

SJS = Stevens-Johnson syndrome; TEN = toxic epidermal necrolysis.
<1>UWortd

This patient's clinical features - acute mucocutaneous disorder with associated systemic
toxicity, following use of sulfonamide antibiotics- are consistent with toxic epidermal
necrolysis (TEN). TEN is an inflammatory hypersensitivity reaction to drugs (especially
sulfonamides, nonsteroidal antiinflammatory drugs, anticonvulsants and allopurinol) or
certain infections (usually Mycoplasma pneumoniae). Clinical features of TEN are
variable, but typically include coalescing erythematous macules, bullae, desquamation,
and mucositis. Patches of skin slide off with light pressure (positive Nikolsky's
sign). Systemic signs are common and may include fever, tachycardia, hypotension,
altered level of consciousness, conjunctivitis, seizures, and coma. The exact
pathogenesis is unknown.
TEN and Stevens-Johnson syndrome (SJS) are related disorders and may be
considered on a continuum of severity. By convention, SJS denotes involvement of
<10% of body surface area (Choice 0), >30% is designated TEN, and 10-30% is
referred to as a SJS/TEN overlap syndrome. The treatment of TEN and SJS is primarily
supportive, with wound care similar to that for burns (and often provided in a designated
burn unit). Aggressive fluid support is often needed due to poor oral intake and profound
cutaneous fluid loss. Secondary infections are common, and antiseptic precautions are
needed.
(Choice A) Erythema multiforme (EM) is characterized by sudden onset erythematous
rash and target lesions and usually occurs after a herpes simplex virus infection. EM
may appear similar to mild SJS/TEN, but bullae and desquamation are less common,
mucosal involvement is rare, and systemic symptoms are not as prominent as in
SJS/TEN.
(Choice B) Exfoliative dermatitis (also known as erythroderma) is a widespread,
scaly eruption of the skin. It may be drug-induced, idiopathic, or secondary to an
underlying dermatological or systemic disease.
(Choice C) Staphylococcal scalded skin syndrome is a syndrome. of acute exfoliation of
skin caused by a toxin that is produced by Staphylococcus aureus. The skin is tender
and warm, with a sandpaper-like, diffuse erythematous rash. Other features include
bullae, positive Nikolsky's sign, facial edema, perioral crusting, and dehydration. It is
usually seen in children less than 6 years of age.
Educational objective:
Toxic epidermal necrolysis (TEN) is a severe syndrome characterized by the sudden
onset of mucocutaneous lesions and systemic signs of toxicity. Typical features include

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 pathogenesis is unknown.
TEN and Stevens-Johnson syndrome (SJS) are related disorders and may be
considered on a continuum of severity. By convention, SJS denotes invo.lvement of
<10% of body surface area (Choice 0), >30% is designated TEN, and 10-30% is
referred to as a SJS/TEN overlap syndrome. The treatment of TEN and SJS is primarily
supportive, with wound care similar to that for burns (and often provided in a designated
burn unit). Aggressive fluid support is often needed due to poo.r oral intake and profound
cutaneous fluid loss. Secondary infections are common, and antiseptic precautions are
needed.
(Choice A) Erythema multiforme (EM) is characterized by sudden onset erythematous
rash and target lesions and usually occurs after a herpes simplex virus infection. EM
may appear similar to mild SJS/TEN, but bullae and desquamation are less common,
mucosal involvement is rare, and systemic symptoms are not as prominent as in
SJS/TEN.
(Choice B) Exfoliative dermatitis (also known as erythroderma) is a widespread,
scaly eruption of the skin. It may be drug-induced, idiopathic, or secondary to an
underlying dermatological or systemic disease.

(Choice C) Staphylococcal scalded skin syndrome is a syndrome of acute exfoliation of

skin caused by a toxin that is produced by Staphylococcus aureus. The skin is tender
and warm, with a sandpaper-like, diffuse erythematous rash. Other features include
bullae, positive Nikolsky's sign, facial edema, perioral crusting, and dehydration. It is
usually seen in children less than 6 years of age.
Educational objective:
Toxic epidermal necrolysis (TEN) is a severe syndrome characterized by the sudden
onset of mucocutaneous lesions and systemic signs of toxicity. Typical features include
an erythematous eruption that rapidly evolves into exfoliation of the skin. It is most often
caused by medications (eg antibiotics, anticonvulsants, allopurinol). TEN and Stevens-
Johnson syndrome (SJS) are similar disorders distinguished by the percentage of
involved skin: SJS involves <10% of the body surface area, TEN involves >30, and
SJS/TEN overlap syndrome involves 10-30%.

References:
1. The current understanding of Stevens-Johnson syndrome and toxic
epidermal necrolysis.

Time Spent: 5 seconds Copyright © UWorld Last updated: (11/19/2016)

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