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

TL;DR

A 48-year-old woman with a severe skin condition and rare lymph node disease was diagnosed with TEN-like ACLE complicated by HNL, highlighting the importance of early detection.

Contribution

This case report presents a rare co-occurrence of TEN-like ACLE and HNL, emphasizing their clinical correlation.

Findings

The patient exhibited TEN-like ACLE with clinical features such as skin sloughing and violaceous macules.

HNL was diagnosed based on biopsy findings and clinical presentation, which is rare in lupus patients.

The patient showed partial improvement with corticosteroids but died from a spontaneous retroperitoneal bleed.

Abstract

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.

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