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

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

Disclosures

January 22, 2019

Certain human leukocyte antigen (HLA) alleles are associated with an increased probability of developing SJS upon exposure to specific drugs; these includes HLA-B*1502 in patients of southeastern Asian ethnicity, which increases the risk of developing SJS while on carbamazepine therapy, and HLA-B*44 in white patients. The HLA-A*0206 and HLA-DQB1*0601 alleles are strongly associated with SJS with ocular involvement.[5]

SJS usually starts with a prodromal phase, including fever, malaise, chills, headaches, nausea, and vomiting. The prodromal phase usually lasts less than 2 weeks and is followed by the eruptive phase. Skin lesions may begin as erythema of the face, neck, and upper chest. They may progress into bullae and skin denudation. The palms, soles, dorsum of the hands, and extensor surfaces are most commonly affected. Lesions that involve the upper gastrointestinal system may cause difficulty eating and drinking, in addition to difficulty swallowing.[6]

The typical skin lesion has the appearance of a target with central purpura. It develops into a bulla and ruptures, leaving an eroded area (Figure 3).

Figure 3.

Ocular involvement is not uncommon; the patient may present with red eyes, foreign body sensation, burning and itching, and gross pain. Ocular signs vary, with a wide spectrum of severity, ranging from simple conjunctivitis, blepharitis, and epithelial defects to more severe signs, such as conjunctival membranes, stromal ulcers, and eventual perforation, which is sight-threatening. If not treated early, these may lead to trichiasis, conjunctival shrinkage, symblepharon, corneal scarring, and eventual blindness.

Mucosal involvement may include erythema, edema, sloughing, blistering, ulceration, and necrosis. Severe systemic complications may ensue if early diagnosis is not made and the offending drug is not promptly discontinued. Scarring and cosmetic deformity, superinfections, esophageal strictures, and renal failure are a few of these eventual complications. As mentioned above, SJS may lead to death.

Early diagnosis is crucial in order to prevent permanent damage and death, especially because SJS at initial presentation my not appear severe. Early diagnosis is achieved by anamnesis and determination of drug exposure or an infection before the onset of symptoms. No specific laboratory studies (other than biopsy) can definitively establish the diagnosis of SJS.

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