Development of Lupus Erythematosus Tumidus During the Course of Systemic Sclerosis

Abstract

A man with systemic sclerosis (SS), manifested by characteristic skin lesions, gastro-esophageal reflux disease, and pulmonary fibrosis producing progressive respiratory failure, and a positive antinuclear antibody consistent with reactivity to fibrillarin, developed skin lesions with the clinical and histological characteristics of lupus erythematosus tumidus (LET) 10 years after the diagnosis of SS. His respiratory failure progressed and he expired from sepsis after tracheal intubation and mechanical ventilation two years after developing LET. The association of SS and LET, not described until now, raises questions about its pathogenesis and its prognostic significance.

Systemic lupus erythematosus presenting as lupus erythematosus tumidus and lupus nephritis: a case report

“…Although systemic symptoms are not commonly observed in patients with LET, one case was reported to have developed systemic symptoms during the course of the disease, leading to a reconsideration of the diagnosis as early-stage SLE. LET has also been reported to occur during the course of Systemic Sclerosis [ 19 ].…”