Desquamation

Question 1

Acute inflammatory skin disease

Acute cutaneous eruption that is brief and self-limiting, but may be
disabling or life-threatening

Sudden onset of systemic erythematous skin lesions with history of recurrence

“Circle within a circle”

Limited to skin and mucous membranes

Answer 1

Erythema Multiforme

Question 2

Approximately what percentage of the Erythema Multiforme cases follow outbreaks of herpes simplex

Answer 2

90%

Question 3

“Circle within a circle”

Red macule or thin papule that expands centrifugally and develops a dusty or necrotic center, creating a target-like pattern

Lesions develop rapidly in crops

Localized to hands and face or generalized (Palms and soles)

Distributed on acral (peripheral) surfaces, but proximal spread to trunk and face not uncommon

Mucous membranes – erosions or ulcers

Bilateral and often symmetric

Answer 3

Erythema Multiforme

Question 4

Erythema Multiforme is a cutaneous reaction to a variety of antigenic stimuli, including what?

Answer 4

Infection
Drugs
Idiopathic

Question 5

What is the pathogenesis of Erythema Multiforme?

Answer 5

Cell-mediated immune reaction

Inflammatory infiltrate is sparse

Mixture of CD4 and CD8 lymphocytes

Keratinocytes are killed due to inflammatory reaction (TNF, cytokines)

Question 6

How is the diagnosis of Erythema Multiforme made?

Answer 6

skin biopsy

Question 7

Which type of EM is described below?

Scattered lesions confined to the skin or when skin lesions are observed in association with limited mucosal involvement

Answer 7

Erythema multiforme minor

Question 8

Which type of EM is described below?

Prominent involvement of at least two of three mucosal sites: oral, anogenital, or conjunctival

Answer 8

Erythema multiforme major

Question 9

What is the treatment for Erythema Multiforme?

Answer 9

Herpes - Treat with oral valacyclovir or famciclovir

Severely ill patients - Systemic glucocorticoids

Discontinue drug if it is the offender

Nutritional and fluid support

Question 10

Acute, life-threatening mucocutaneous reaction characterized by extensive necrosis and detachment of the epidermis

Characterized by toxicity and involvement of two or more mucosal surfaces

Often oral and conjunctiva

Cell-mediated cytotoxic reaction

Cause - Drug induced (most commonly), idiopathic

Answer 10

Stevens-Johnson Syndrome

Question 11

A progression of SJS to generalized epidermal detachment

When there is >30% body surface area (BSA) skin loss

Begins as generalized erythema, conjunctivitis, stomatitis, epidermal shearing from friction

Complications: pneumonia, septicemia, fluid and electrolyte loss

Answer 11

Toxic Epidermal Necrolysis

Question 12

What is the number one cause of death in TEN?

Answer 12

infection/sepsis

Question 13

What are some risk factors for developing SJS/TEN?

Answer 13

SLE
HLA-B12
HIV/AIDS

Question 14

What is the distribution of SJS?

Answer 14

Widely distributed with prominent involvement of trunk and face

<10% epidermal detachment

Question 15

What is the distribution of TEN?

Answer 15

Generalized, universal

there is >30% body surface area (BSA) skin loss

Question 16

In SJS, what percentage of patients have conjunctival lesions?

Answer 16

85% have conjunctival lesions

Question 17

What is the treatment for SJS/TEN?

Answer 17

Early diagnosis and withdrawal of suspected drugs are very important!

Supportive care - ICU: IV fluids and electrolytes, Treat complications

Systemic glucocorticoids: Early – recommended, Late – contraindicated

High dose IV immunoglobulins: Early on in the disease