De novo Presentation of Systemic Lupus as Bullous Erythematosus: A Case Report.

Abstract

Background: Systemic Lupus Erythematosus (SLE) (C1) is a disease with multi-organ involvement that can have a variety of cutaneous manifestations in 76% of cases during the disease. Less than 1% of these patients are diagnosed with confirmed bullous systemic lupus erythematosus (C1). Given the wide differential diagnosis of a bullous lesion, it is imperative to reach a conclusive diagnosis as it can have a direct impact on the course of management of the disease. Here, we present a case of SLE with a de novo presentation of bullous lesions. Throughout the length of the report, we will go through the protracted clinical course of the patient, followed by a clinically relevant discussion of the condition.

Case presentation: The case describes the presentation of a young African female of low socio-economic status with first-ever eruption of bullous lesions on her trunk and groin. The lesions progressed to involve the face. A biopsy was taken, and the patient was started on dapsone and hydroxychloroquine. She initially responded well but soon developed Steven Johnson syndrome in reaction to dapsone. In the meantime, a biopsy and hematological work-up confirmed a diagnosis of Bullous SLE. The patient was started on methotrexate, to which she initially responded well but developed methotrexate-induced cytopenia. This was followed by initiation of mycophenolate, to which the patient responded very well and was subsequently discharged on the same. At the time of discharge, all lesions healed, and the hematological workup remarkably improved.

Conclusion: All patients with bullous lesions should be evaluated for bullous SLE. A definitive diagnosis will chart the course of management. Multiple drug options are available, and there is no single hierarchy of medicines that will suit all. Sometimes, multiple modalities need to be tried before the patient achieves clinical remission and then can be continued on the same.