Calciphylaxis with pseudoxanthoma elasticum-like changes: A case series

Calciphylaxis and pseudoxanthoma elasticum (PXE) are rare, clinically distinct disorders that share a common feature of cutaneous calcification and that vary widely in their cutaneous presentation.

We conducted a descriptive, retrospective review of biopsy specimens collected over a 2-year period. Only specimens with a histologic and clinical diagnosis of calciphylaxis were included in the review. Specimens were then histologically examined for features of PXE in the dermis and/or subcutaneous fat, utilizing hematoxylin and eosin staining. Von Kossa and Verhoeff-Van Gieson special stains were also performed to examine calcification and elastic fibers, respectively.

We reviewed 13 biopsy specimens from 9 patients with known clinical and histologic evidence of calciphylaxis, both uremic and non-uremic types. Upon re-examination, we found that 46.2% (n = 6/13) of the specimens showed concomitant PXE-like changes uniquely localized to the subcutaneous fat.

The presence of PXE-like changes in the subcutis may heighten suspicion for a diagnosis of calciphylaxis in the appropriate clinical setting and be helpful when classic histologic features are subtle or absent.