Adult-onset Atypical Stevens-Johnson Syndrome with HLA-A*11:01 and HLA-A*31:01 Alleles

ABSTRACT

Purpose@#We report a case of adult-onset atypical Stevens-Johnson syndrome (SJS) in which only the oral and ocular mucosae were involved, without skin lesions. Case summary: A 33-year-old man was referred to our institute with recent severe oral mucositis and bilateral conjunctival hyperemia and pseudomembrane formation, which occurred 5 days after taking cold medicine, including a non-steroidal anti-inflammatory drug, acetaminophen and cefaclor, for pharyngitis with fever. At the initial visit, the patient had an epithelial defect of the bulbar and tarsal conjunctiva in both eyes. Conservative treatment using topical eye drops was started, with suspicion of atypical SJS. In addition, HLA-A alleles were typed to assess possible genetic susceptibility to the relevant drugs, and HLA-A*1101 and HLA-A*3101 alleles were confirmed. Immunoglobulin (Ig) G against Mycoplasma pneumoniae was positive and IgM was negative at 10 months after the initial onset of disease. One year after the disease onset, autologous serum eye drops, oral and topical steroids, 0.1% cyclosporine eye drops, and antibiotic eye drops were maintained and the corneal and conjunctival erosions were much improved. @*Conclusions@#Although atypical SJS is very rare, it is important to suspect it early by taking a history of any preceding medication or possible concurrent mycoplasma pneumonia, because atypical SJS may affect the oral and ocular mucosae without skin lesions.