A Rare Presentation of Toxic Epidermal Necrolysis – Like Acute Systemic Lupus Erythematosus

Brandon T Beal,Steven D Billings,Shilpi Khetarpal,Taryn Blaha,David D Xiong,Sarah H Schneider,Anthony P Fernandez

doi:10.25251/skin.2.2.9

Abstract

A 25-year-old woman with a history of systemic lupus erythematosus (SLE) was transferred from an outside hospital with a worsening painful generalized rash and oral ulcerations for the prior 3 weeks due to concern for Stevens-Johnson Syndrome / toxic epidermal necrolysis (TEN). Exam revealed denuded erythematous plaques covering over 80% of the patient’s body surface area and a 4.2 x 2.6 cm ulcerated plaque of the superior hard palate. Histology demonstrated parakeratosis and compact hyperkeratosis overlying an atrophic epidermis with vacuolar interface change, prominent keratinocyte dyskeratosis, and a thickened basement membrane zone (BMZ). The superficial dermis had a mild predominantly lymphocytic perivascular infiltrate and superficial dermal mucin deposition. Direct immunofluorescence was positive for IgG (granular, BMZ), C3 (granular, BMZ), and IgA was negative. Labs were remarkable for positive ANA (1:160), ds-DNA, anti-Smith, anti-RNP, low C3/C4, with negative anti-SSA/SSB. The clinicopathological correlation was most consistent with the diagnosis of TEN-like acute systemic lupus erythematosus (TEN-like ASLE). Our patient improved with treatment for her ASLE. This case highlights a challenging clinicopathologic differential diagnosis.

Highlights

Acute systemic lupus erythematosus presenting as toxic epidermal necrolysis (TEN-like ASLE) is rare with less than 25 reported cases. 1 Differentiating ASLE and TEN can be difficult, as both share clinical and histological findings such as diffuse desquamation, mucosal erosions, and keratinocyte necrosis
A lesional punch biopsy was performed showing areas of parakeratosis and compact hyperkeratosis overlying an atrophic epidermis with vacuolar interface change and prominent keratinocyte dyskeratosis and apoptosis
Direct immunofluorescence was positive for IgG and C3, SKIN

Summary

INTRODUCTION

Acute systemic lupus erythematosus presenting as toxic epidermal necrolysis (TEN-like ASLE) is rare with less than 25 reported cases. 1 Differentiating ASLE and TEN can be difficult, as both share clinical and histological findings such as diffuse desquamation, mucosal erosions, and keratinocyte necrosis. Acute systemic lupus erythematosus presenting as toxic epidermal necrolysis (TEN-like ASLE) is rare with less than 25 reported cases. Prodromal systemic symptoms (fatigue, myalgias, and polyarthritis), positive ANA and ENA, photodistributed eruption, and a prolonged disease course characterize ASLE, while an identifiable trigger and more acute onset (less than 9 days) favor TEN. A generalized rash and oral ulcerations for the prior 3 weeks due to concern for StevensJohnson Syndrome / toxic epidermal necrolysis. Prior to admission, she noted a diffuse and painful rash, large oral ulcerations, fatigue, and muscle/joint pain. These findings were most consistent with an interface dermatitis (Figure 3). Our patient improved with a loading dose of hydroxychloroquine, 200mg three times daily for three months following by 5mg/kg/day thereafter, methylprednisone 1g IV every 24 hours for the first three days and she was transitioned to oral prednisone 60mg daily which was tapered over approximately 6 months, mycophenolate mofetil 1g twice daily, and dapsone 100mg daily

DISCUSSION

Findings

CONCLUSION