Document Type

Article

Publication Date

10-1-2023

Comments

This article is the author’s final published version in JAAD Case Reports, Volume 40, October 2023, Pages 19-22.

The published version is available at https://doi.org/10.1016/j.jdcr.2023.07.015. Copyright © 2023 by the American Academy of Dermatology, Inc. Published by Elsevier Inc.

Abstract

Introduction

Calcinosis cutis, a disorder in which calcium salts deposit in skin and subcutis, is categorized into five subtypes: dystrophic calcification, metastatic calcification, idiopathic calcification, iatrogenic calcification, and calciphylaxis.

Dystrophic calcification, the most common subtype, typically results from local tissue damage1,2 and is proposed to be caused by the release of phosphate binding proteins by necrotic cells in response to tissue damage, inflammation, or hypoxia.2 The condition often presents with nontender nodules of the skin or subcutis and normal serum calcium.

Calciphylaxis is believed to be caused by impaired inhibition of calcification in the microvasculature.3 A deficiency in carboxylated matrix Gla protein, a vitamin K dependent inhibitor of vascular calcification, has been associated with calciphylaxis.3 Conditions resulting in vitamin K deficiency, including Warfarin usage and end stage renal disease, have been implicated.3 Several causes of nonuremic calciphylaxis have been reported, notably in association with alcoholic cirrhosis.1,4,5 Additional risk factors include hypercalcemia, hyperphosphatemia, and hyperparathyroidism.3

Intramural vascular calcification of small to medium-sized vessels, typically of the dermis and subcutaneous fat, is a key pathologic diagnostic criterion of calciphylaxis.1 Following medial calcification, subintimal fibroplasia and thrombosis result in vascular occlusion, ischemia, inflammation, and necrosis of surrounding tissue.3 Consequently, calciphylaxis is associated with severely painful lesions.3 Diagnosis requires clinicopathologic correlation as diagnostic histopathological findings of intramural calcification of small vessels and vasculopathy can be subtle.

The following case underscores how variations in the clinicopathologic presentation of dystrophic calcification and calciphylaxis can create diagnostic challenges.

Creative Commons License

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 License.

PubMed ID

37675067

Language

English

Download

Included in

Dermatology Commons

Share

COinS