Toxic Epidermal Necrolysis-Like Acute Cutaneous Lupus Erythematosus With Histiocytic Necrotizing Lymphadenitis: A...

Toxic epidermal necrolysis-like acute cutaneous lupus erythematosus (TEN-like ACLE) is an acute, life-threatening manifestation of ACLE. Histiocytic necrotizing lymphadenitis (HNL) is a rare disease of lymph nodes that presents with fever and lymphadenopathy and is generally self-resolving. We report a case of a 48-year-old female who presented to the emergency department (ED) with fever and arthralgias in the setting of 30 lbs weight loss over two months. She had self-discharged and re-presented to the ED three weeks later with widespread, painful skin sloughing. On exam, the patient had scattered, violaceous macules coalescing into patches, flaccid bullae, and erosions on her scalp, face, upper central chest, shoulders, arms, and lower legs. She had no oral, ocular, or genital mucosal involvement. Biopsy showed vacuolar interface dermatitis with granular deposits of IgM, IgG, and C3 along the basement membrane zone. A diagnosis of TEN-like ACLE complicated by HNL was made, given the clinical picture and biopsy findings. The patient showed improvement with topical triamcinolone and oral corticosteroid but ultimately passed away secondary to a spontaneous retroperitoneal bleed. This case highlights the clinical features and management of TEN-like ACLE, as well as its correlation with HNL, to facilitate earlier detection and intervention.