Bullous Systemic Lupus Erythematosus

Akm Humayon Kabir,Abu Baker Siddique,Arman Hussain,Md Moniruzzaman,Md Mujibur Rahman,Sabrina Rahman,Sk Jakaria Been Sayeed,Uzzal Mallik

doi:10.3329/jom.v19i2.37233

Akm Humayon Kabir, Abu Baker Siddique + Show 6 more

Open Access

https://doi.org/10.3329/jom.v19i2.37233

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Abstract

Bullous systemic lupus erythematosus (BSLE) is extremely rare but distinct disease, characterized by vesicobullous skin eruptions in systemic lupus erythematosus (SLE). It can develop either before or after a diagnosis of SLE has been established. BSLE is characterized by a dermatitis herpetiformis-like histology and an autoimmunity to type VII collagen. It must be differentiated from other autoimmune vesicobullous diseases such as epidermolysis bullosa acquisita, dermatitis herpetiformis, linear IgA disease, and bullous pemphigoid. Its important to combine clinical, histological, and immunofluorescence findings to establish a diagnosis of BSLE. We report a case of BSLE to illustrate and emphasize the need for an integrative diagnostic approach.J MEDICINE JUL 2018; 19 (2) : 123-125

Highlights

Bullous systemic lupus erythematosus (BSLE) is a rare autoimmune blistering disorder that typically manifests as an acute vesiculobullous eruption in a patient with known systemic lupus erythematosus (SLE)
BSLE patients produce autoantibodies that recognize type VII collagen, a major component of anchoring fibrils, which play an important role in dermo- epidermal adhesion
The skin eruption comprises bullae arising either from areas affected by diffuse erythema or on an urticarial base resembling bullous pemphigoid, or as grouped vesicular lesions mimicking dermatitis herpetiformis.[6,7]

Summary

Introduction

Bullous systemic lupus erythematosus (BSLE) is a rare autoimmune blistering disorder that typically manifests as an acute vesiculobullous eruption in a patient with known systemic lupus erythematosus (SLE). Case summary: A 20 years old female, married presented with multiple painful non pruritic tense blister on her face, neck, front and back of the trunk, both upper & lower extremities. These lesions were eventually ruptured and healed up spontaneously without scarring leaving multiple hypopigmented & hyperpigmented patches (Figure 1). ANA (9.9IU/ml) & Anti ds-DNA (33.9IU/ml) were strongly positive Her 24 hour UTP was normal (0.20 g/day). As blister appeared persistently we add Dapsone (50mg/day) that made striking response

Discussion

Findings

Conclusion