Overlap of Toxic Epidermal Necrolysis (TEN)-Like Cutaneous Lupus and Rowell Syndrome in Systemic Lupus Erythematosus:...

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a broad range of systemic and cutaneous manifestations. Among its rare dermatologic variants are toxic epidermal necrolysis (TEN)-like acute cutaneous lupus erythematosus (ACLE), marked by erythema multiforme-like lesions and a distinct immunologic profile, and Rowell syndrome (RS). Unlike drug-induced TEN, both are autoimmune conditions. In this patient, the two entities did not coexist, but their striking clinical resemblance posed a significant diagnostic challenge. A 49-year-old Indonesian woman with a previously unremarkable medical history presented with a diffuse erythematous eruption beginning on the face and then spreading downward to involve the trunk and extremities, mucocutaneous involvement, and reticulated purpura. Laboratory investigations revealed severe hyponatremia, renal impairment, anemia, thrombocytopenia, low complement levels, ANA, anti-dsDNA, anti-Sm, anti-Ro/SSA, and anti-La/SSB antibodies, and all had strongly positive serology. Skin biopsy findings included full-thickness epidermal necrosis with interface dermatitis, thereby confirming the diagnosis of TEN-like ACLE. The patient was treated with systemic immunosuppression with agents including high-dose corticosteroids and mycophenolate mofetil together with supportive care, resulting in clinical stabilization. This uncommon and diagnostically challenging cutaneous manifestation of SLE carries significant clinical implications, particularly when distinguishing between TEN-like ACLE, RS, and drug-induced TEN. Prompt identification and timely initiation of immunosuppressive therapy are critical to reducing morbidity and preventing potential mortality. Additionally, we provide a comprehensive review of previously reported cases and discuss current diagnostic strategies and therapeutic approaches.