A 2-Year-Old-Boy With an Alarming Facial Rash

Buraa Kubaisi, MD; Nakhoul Nakhoul, MD; C. Stephen Foster, MD


January 22, 2019


Stevens-Johnson syndrome (SJS) is an immune complex-mediated hypersensitivity reaction in the skin and mucous membranes. SJS and toxic epidermal necrolysis are similar, with variable involvement. When less than 10% of body surface area is involved, the condition is considered SJS; when more than 30% of body surface area is involved, it is considered TEN. When 10%-30% of body surface area is involved, it is considered overlapping SJS/TEN. SJS can involve the skin, eyes, and nose and the gastrointestinal, respiratory, and genitourinary systems, leading to severe morbidity and, in some instances, eventual death.[1]

The pathophysiology of SJS has been thoroughly studied. It involves antigen presentation and production of tumor necrosis factor alpha by dendrocytes, activating T lymphocytes that induce epidermal cell apoptosis. The death of keratinocytes causes separation of the epidermis from the dermis. The dying cells provoke recruitment of more chemokines, leading to a vicious cycle of more inflammation and more cell death with extensive epidermal necrolysis. This is compatible with the histopathologic findings in this patient, in whom necrosis of the epidermis was seen, along with mild dermal inflammatory cell infiltration.[2]

SJS can affect persons of all races and ages and both sexes. Various etiologies have been implicated in SJS, including the following[3,4]:

  • Drugs (most commonly recognized cause in adults): antibiotics, anticonvulsants, nonsteroidal anti-inflammatory drugs, and analgesics

  • Infections: Viral (herpes simplex virus, AIDS), bacterial (streptococcal, mycobacterial), fungal species (coccidioidomycosis, histoplasmosis)

  • Cancer

  • Autoimmune conditions

  • Trauma

  • Vaccination

  • Idiopathic (25%-50% of cases)

The differential diagnosis of SJS includes exfoliative dermatitis, toxic shock syndrome, staphylococcal scalded skin syndrome, bullous systemic lupus erythematosus, epidermolysis bullosa acquisita, and paraneoplastic pemphigus.


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