A Case of Steven Johnson Syndrome-Toxic Epidermal Necrolysis Overlap with Systemic Lupus Erythematosus

Authors: Leah Antoinette M. Caro-Chang, Sarah Faye V. Obbus, Claudine Yap-Silva, Eileen Liesl A. Cubillan, Georgina C. Pastorfide

Abstract

Introduction: We report a case of Steven Johnson Syndrome-Toxic Epidermal Necrolysis (SJS-TEN) overlap in a patient with systemic lupus erythematosus (SLE). Identified drugs were amoxicillin-clavulanic acid and ibuprofen however, connective tissue diseases such as SLE contributed to this as well.

Case summary: A 20-year-old-female presented with multiple erythematous to violaceous plaques, crusted erosions, and some bullae. Two months prior to admission, the patient was given amoxicillin-clavulanic acid and ibuprofen for otitis media. On the sixth day of medications, she developed facial swelling and erythema. She consulted several physicians and diagnosed to have measles and a hypersensitivity reaction warranting more medications such as cetirizine, paracetamol, methylprednisolone, hydroxyzine, fexofenadine and ranitidine but facial erythema persisted. Two weeks before admission, she developed a blister on her forehead on top of the facial erythema. Cutaneous lupus was considered but cetirizine was given for the drug reaction. After a week, the erythema and blisters spread to the trunk and extremities, undocumented fever, malaise and purulent discharge on both eyes. She was managed as a case of SJS-TEN overlap (26% BSA involvement, SCORTEN 2) and immunologic work-up for SLE was positive. IVIg (30mg) was administered and hydroxychloroquine was also started. The patient developed blisters and more erythematous plaques despite IVIg. Biopsy of the bulla was consistent with bullous LE and the lesions on the legs showed interface dermatitis consistent with lupus erythematosus. The patient was then started on prednisone and no new lesions developed.

Conclusion: SJS/TEN and SLE are inflammatory dermatoses that have keratinocyte apoptosis in their pathophysiology. A meticulous drug-history coupled with the proper work-up, including skin biopsy, may help differentiate these two conditions or identify their coexistence leading to the proper treatment.

 

Citation

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Keywords

Stevens-Johnson Syndrome, Toxic Epidermal Necrolysis, Systemic Lupus Erythematosus

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