Histopathology of calciphylaxis: Cohort study with clinical correlations

Tiffany Y. Chen, Julia Lehman, Lawrence E. Gibson, Christine M. Lohse, Rokea A. El-Azhary

Research output: Contribution to journalArticle

26 Citations (Scopus)

Abstract

Calciphylaxis is a rare, painful, and life-threatening condition with a high mortality rate. Although the etiology of calciphylaxis is not well understood, it has been proposed that calcium deposition within and around subcutaneous vessels restricts blood flow chronically, thereby predisposing the patient to acute pannicular and dermal thrombosis. Given increasing recognition of the role of hypercoagulability in calciphylaxis, this retrospective cohort study sought to evaluate the presence of thromboses and dermal angioplasia in calciphylaxis. Moreover, we aimed to validate previous observations about the histopathology of calciphylaxis compared with skin biopsies from patients with end-stage renal disease but without calciphylaxis. After a meticulous clinical chart review, we assessed the corresponding skin biopsies for the presence of vessel calcification, thromboses, and dermal angioplasia in skin biopsies from patients with calciphylaxis (n = 57) and compared with those from patients with end-stage renal disease but without calciphylaxis (n = 26). Histopathologic findings were correlated with clinical features such as chronic kidney disease, diabetes, or associated malignancy. Our results validated a prior observation that calciphylaxis was significantly more likely to show calcification of dermal vessels and diffuse dermal thrombi. This study reports the frequent finding of dermal angioplasia, a potential marker of chronic low-grade ischemia, as another frequent microscopic finding in calciphylaxis. Among cases of calciphylaxis, histopathologic changes in patients with chronic kidney disease were indistinguishable from those in patients without chronic kidney disease, thereby implying a final common pathogenic pathway in both uremic and nonuremic calciphylaxis. In future, larger, prospective studies may be useful in validating these findings.

Original languageEnglish (US)
Pages (from-to)795-802
Number of pages8
JournalAmerican Journal of Dermatopathology
Volume39
Issue number11
DOIs
StatePublished - Nov 1 2017

Fingerprint

Calciphylaxis
Cohort Studies
Skin
Thrombosis
Chronic Renal Insufficiency
Biopsy
Chronic Kidney Failure
Thrombophilia

Keywords

  • Calciphylaxis
  • Histopathology
  • Panniculitis
  • Vasculopathy

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Dermatology

Cite this

Histopathology of calciphylaxis : Cohort study with clinical correlations. / Chen, Tiffany Y.; Lehman, Julia; Gibson, Lawrence E.; Lohse, Christine M.; El-Azhary, Rokea A.

In: American Journal of Dermatopathology, Vol. 39, No. 11, 01.11.2017, p. 795-802.

Research output: Contribution to journalArticle

Chen, Tiffany Y. ; Lehman, Julia ; Gibson, Lawrence E. ; Lohse, Christine M. ; El-Azhary, Rokea A. / Histopathology of calciphylaxis : Cohort study with clinical correlations. In: American Journal of Dermatopathology. 2017 ; Vol. 39, No. 11. pp. 795-802.
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