Abstract
Pseudoxanthoma elasticum (PXE) is a rare, inherited connective tissue disorder characterized by the accumulation of calcified elastic fibers. This mineralization can affect elastic fibers in the skin, eyes, cardiovascular tissue, and less commonly, the gastrointestinal tract, leading to various clinical presentations such as skin changes, vision problems, or claudication. Histopathologic features of PXE include thickened, calcified elastic fibers within the reticular dermis. There have been reported cases of inflammatory skin diseases that demonstrate identical or highly resemblant PXE histopathologic changes without clinical manifestations of PXE. We herein report a case series of four patients diagnosed with panniculitis associated with PXE-like changes without any clinical features of PXE. The clinical significance of these findings remain uncertain due to limited literature. In addition to reporting our institutional experience on four clinical cases, we will discuss the current understanding of the proposed pathogenesis of panniculitis with PXE features and encourage further investigation to elucidate the clinical significance of these findings.
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