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Skin-nontumor

Blistering disorders

Stevens-Johnson syndrome


Reviewer: Mowafak Hamodat MB.CH.B, MSc., FRCPC, Eastern Health, St. Johns (Canada) (see Reviewers page)
Revised: 9 July 2011, last major update July 2011
Copyright: (c) 2002-2011, PathologyOutlines.com, Inc.

Definition
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● Severe adverse cutaneous drug reaction that predominantly involves the skin and mucous membranes (Orphanet J Rare Dis 2010 Dec 16;5:39)
● Systemic form of erythema multiforme with fever

Terminology
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● Toxic epidermal necrolysis: variant with full thickness epidermal necrosis, but with intact cornified layer, subepidermal bullae and sloughing of skin/mucosal epithelium; analogous to severe burn

Clinical features
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● Common in children
● Often involves lips, oral mucosa or other mucosa with erosions and hemorrhagic crusts
● Infection of affected areas may cause life threatening sepsis

Clinical images
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Stevens-Johnson syndrome

Micro description
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● Full thickness epidermal necrosis with separation of epidermis from dermis
● Necrotic keratinocytes at edge of bullae

Differential diagnosis
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Staphylococcal scalded skin syndrome: similar clinically, but plane of cleavage is in granular layer
Necrolytic migratory erythema: also called glucagonoma syndrome has superficial epidermal necrosis

Additional references
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eMedicine

End of Skin-nontumor > Blistering disorders > Stevens-Johnson syndrome


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