Findings of osseous sclerotic bodies: a unique sequence of cutaneous bone formation in nephrogenic systemic fibrosis

Abstract

Nephrogenic systemic fibrosis (NSF) is a progressive and potentially fatal fibrosing skin disorder found mainly in patients with renal insufficiency. NSF is characterized by thickened and hyperpigmented skin lesions with or without systemic involvement. In essentially all patients, this disease entity has been associated with the administration of gadolinium contrast agents for imaging purposes. Microscopic recognition of this entity remains challenging, as the diagnosis is based on various clinical and histopathologic features that overlap with other fibrosing disorders. No single feature is absolutely specific for NSF. We report a finding of osseous sclerotic bodies with elastin trapping appearing on histopathology in the clinical setting of NSF with hemodialysis-dependent renal failure. Our report of an additional attribute indicative of NSF may aid in making the diagnosis.

Brain gadolinium deposition after administration of gadolinium-based contrast agents

“…In 2015, Gathings et al [59] reported two cases of gadolinium-associated skin plaques in patients without severe renal dysfunction. This plaque was reported as the sclerotic body that had been linked to NSF [60][61][62]. The gadolinium-associated plaques caused only mild symptoms, and were associated with few problems clinically.…”

Lollipop lesions in nephrogenic systemic fibrosis mimicking a deep fungal infection

“…Histopathologic findings are not entirely specific and include thickened collagen bundles with increased numbers of CD34‐positive and procollagen‐positive spindle cells with associated mucin deposition . The term lollipop lesion was recently proposed to describe sclerotic amorphous eosinophilic bodies with entrapped elastic fibers , . Lollipop lesions are believed to be highly specific for NSF.…”

“…Therefore, identifying histologic features that may be unique to NSF would be valuable in discriminating between two pathologically similar conditions. Clues to the diagnosis of NSF include osteoclastic giant cells, dystrophic calcification, osseous metaplasia, elastolysis and sclerotic bodies , …”

“…The name was derived from the fact that a stick‐like elastic fiber protruding from a rounded sclerotic body simulates the configuration of a lollipop. Lollipop lesions are believed to be very specific for NSF and thus are diagnostically helpful , …”

“…1 The term lollipop lesion was recently proposed 2 to describe sclerotic amorphous eosinophilic bodies with entrapped elastic fibers. 1,4,5 Lollipop lesions are believed to be highly specific for NSF. Herein, we report a case of NSF that histopathologically mimicked a deep fungal infection.…”