Stevens-Johnson syndrome/toxic epidermal necrolysis
@differential_diagnosis1Stevens-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. Mucous membranes are affected in over 90 percent of patients, usually at two or more distinct sites (ocular, oral, and genital).
Nikolsky's Sign in a patient with Toxic Epidermal Necrolysis (TEN) π
DIFFERENTIAL DIAGNOSIS π
π·οΈ Erythema multiforme β Erythema multiforme usually presents with typical target lesions or raised, atypical, targetoid lesions that are predominantly located on the extremities. Bullae and epidermal detachment are usually limited and involve less than 10 percent of the body surface area (BSA). In contrast with SJS/TEN, erythema multiforme is associated with infection with herpes simplex virus in approximately 90 percent of cases and only rarely with drugs.
π·οΈ Erythroderma and erythematous drug eruptions β The generalized and symmetric, maculopapular erythema of a drug eruption can mimic early SJS/TEN. However, exanthematous drug eruptions lack mucosal involvement and the prominent skin pain of TEN. Histology shows only a mild interface dermatitis with a perivascular, inflammatory infiltrate of lymphocytes, neutrophils, and eosinophils.
π·οΈ Acute generalized exanthematous pustulosis β Severe cases of acute generalized exanthematous pustulosis (AGEP) may be difficult to differentiate from SJS/TEN. AGEP typically develops within a few days of exposure to the offending drug, most often a beta-lactam antibiotic, and resolves without treatment in one to two weeks after drug discontinuation. The histologic hallmark of AGEP is a spongiform, subcorneal, and/or intraepidermal pustule.
π·οΈ Generalized bullous fixed drug eruption β Generalized bullous fixed drug eruption is an extremely rare form of fixed drug eruption characterized by widespread red or brown macules or plaques with overlying, large, flaccid bullae. In contrast with SJS/TEN, mucosal involvement is usually absent. Resolution generally occurs in one to two weeks after drug discontinuation.
π·οΈ Phototoxic eruptions β Severe phototoxic eruptions may be confused with SJS/TEN. Important clues to the correct diagnosis include recent sun exposure, known phototoxic properties of certain medications, and location of the lesions on sun-exposed areas.
π·οΈ Staphylococcal scalded skin syndrome β Staphylococcal scalded skin syndrome (SSSS) is caused by epidermolytic toxins produced by certain strains of staphylococci and is usually seen in neonates and young children. SSSS presents with generalized erythema rapidly followed by the development of flaccid blisters and desquamation. The mucous membranes are not involved. Histology reveals sloughing of only the upper layers of the epidermis, in contrast with the subepidermal split with full-thickness epidermal necrosis observed in SJS/TEN.
π·οΈ Paraneoplastic pemphigus β Paraneoplastic pemphigus is a rare disorder that can represent the initial presentation of a malignancy or occur in a patient with a known neoplastic process, such as non-Hodgkin lymphoma in adults or Castleman's disease in children. Patients may develop severe mucocutaneous disease with ocular and oral blisters and skin lesions.
π·οΈ Linear IgA bullous dermatosis β Linear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disease that may mimic TEN. Histology reveals a subepidermal blister with an underlying, neutrophil-predominant, dermal infiltrate. Direct immunofluorescence shows linear deposits of IgA along the basement membrane.
π·οΈ Chikungunya fever β An atypical, SJS/TEN-like form of chikungunya fever characterized by fever and generalized, vesicobullous eruption and superficial erosions has been reported in infants and young children. However, in contrast with SJS/TEN, mucosal involvement is generally absent, and the resolution of the skin manifestations occurs in most cases in 4 to 10 days.
Drugs associated with Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN)
Strongly associated*
π Allopurinol
π Lamotrigine
π Sulfamethoxazole
π Carbamazepine
π Phenytoin
π Nevirapine
π Sulfasalazine
π Other sulfonamides
π Oxicam NSAIDs (piroxicam, tenoxicamΒΆ)
π Phenobarbital
π EtoricoxibΒΆ
AssociatedΞ
π Diclofenac
π Doxycycline
π Amoxicillin/ampicillin
π Ciprofloxacin
π Levofloxacin
π Amifostine
π Oxcarbazepine
π Rifampin (rifampicin)
Suspected association/lower riskβ
π Pantoprazole
π Glucocorticoids
π Omeprazole
π TetrazepamΒ§
π Dipyrone (metamizole)ΒΆ
π Terbinafine
π Levetiracetam
NSAID: nonsteroidal anti-inflammatory drug; RegiSCAR: International Registry of Severe Cutaneous Adverse Reactions.
* Significant association in case-control studies with a lower limit of the confidence interval β₯5 and unpublished data from the RegiSCAR.
ΒΆ Etoricoxib, tenoxicam, and dipyrone are NSAIDs available in some countries outside of North America.
Ξ Significant association in case-control studies with a lower limit of the confidence interval <5.
β Some cases with plausible causality in medical literature and/or RegiSCAR European Registry.
Β§ A benzodiazepine is available in some countries outside of North America.
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