Dataset Information

Correlation between clinical and pathological features of cutaneous calciphylaxis.

ABSTRACT: Calciphylaxis is a rare and life-threatening disease that classically manifests with painful skin lesions. It occurs mainly in patients with end-stage renal disease (ESRD) treated with dialysis, has poor outcomes, and has no FDA-approved treatment. Our cohort study aims to examine the clinical and pathological features of calciphylaxis and investigates the correlation between cutaneous clinical manifestations and histopathological findings. Data from 70 calciphylaxis patients who were evaluated at the Massachusetts General Hospital between January 2014 and April 2018 were collected from the institutional electronic database. The median age was 58 years (interquartile range [IQR]: 49-69 years), 60% were women, and 73% were of white race. Most (74%) patients reported severe pain at the time of calciphylaxis diagnosis with a median pain intensity score of 8/10 (IQR: 6-10) on a 0-10 pain scale. The median time from symptom onset to clinical diagnosis was 9 weeks (IQR: 6-16 weeks). The majority (87%) of patients presented with open necrotic wounds (advanced stage lesion) at the time of diagnosis. Common cutaneous clinical features included ulceration (79%), induration (57%), and erythema (41%), while common pathological features included cutaneous microvascular calcification (86%) and necrosis (73%). The presence of fibrin thrombi in skin biopsies was associated with pain severity (p = 0.04). The stage of a skin lesion positively correlated with the presence of necrosis on histological analyses (p = 0.02). These findings have implications for improving understanding of calciphylaxis origins and for developing novel treatments.

SUBMITTER: Dutta P

PROVIDER: S-EPMC6564670 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature

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Correlation between clinical and pathological features of cutaneous calciphylaxis.

Dutta Puja P Chaudet Kristine M KM Nazarian Rosalynn M RM Kroshinsky Daniela D Nigwekar Sagar U SU

PloS one 20190613 6

Calciphylaxis is a rare and life-threatening disease that classically manifests with painful skin lesions. It occurs mainly in patients with end-stage renal disease (ESRD) treated with dialysis, has poor outcomes, and has no FDA-approved treatment. Our cohort study aims to examine the clinical and pathological features of calciphylaxis and investigates the correlation between cutaneous clinical manifestations and histopathological findings. Data from 70 calciphylaxis patients who were evaluated ...[more]

PMID: 31194797

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Project description:Though widely used to assess pathological scars, the modified Vancouver Scar Scale (mVSS) is neither convenient nor objective. Shear wave elastography (SWE) is used to evaluate the stiffness of pathological scars. We aimed to determine the correlation between mVSS score and elastic modulus (EM) measured by SWE for pathological scars. Clinical information including ultrasound (US) results of the enrolled patients with pathological scars was analyzed. The clinical severity of the pathological scars was evaluated by mVSS. Skin stiffness, as represented by EM, was calculated using SWE. The average EM of the whole scar (EMWHOLE), hardest part of the scar (EMHARDEST), and normal appearance of the skin around the scar (EMNORMAL) were also recorded. Enrolled in this study were 69 pathological scars, including 28 hypertrophic scars and 41 keloids. The univariable regression analyses showed that the EM of pathological scars was closely related to mVSS score, while the linear multivariable regression analyses showed no significantly correlation. Curve fitting and threshold effect analysis revealed that when EMWHOLE was less than 166.6 kPa or EMHARDEST was less than 133.07 kPa, EM was positively correlated with mVSS score. In stratified analysis, there was no significant linear correlation and threshold effect between EMWHOLE and mVSS score in hypertrophic scars or keloids. However, the fully adjusted smooth curves presented a linear association between mVSS score and EMHARDEST in keloids (the adjusted β [95% CI] was 0.010 [0.001, 0.018]), but a threshold and nonlinear association were found in hypertrophic scars. When EMHARDEST was less than 156.13 kPa, the mVSS score increased along with the hardest scar part stiffness; the adjusted β (95% CI) was 0.024 (0.009, 0.038). In conclusion, EM of pathological scars measured by SWE were correlated with mVSS within a threshold range, and showed different association patterns in hypertrophic scars and keloids.

Dataset Information

Correlation between clinical and pathological features of cutaneous calciphylaxis.

Publications

Correlation between clinical and pathological features of cutaneous calciphylaxis.

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