Nephrogenic systemic fibrosis (NSF) is a disease marked by skin stiffening in the setting of a patient with kidney disease. The disease has been linked with MRI Contrast Agents. There is no evidence to suggest that patients without kidney disease can develop NSF.
Our laboratory investigates cases of possible NSF (examining clinical records and tissue slides) to try to confirm the diagnosis. Once confirmed, we interview patients, conduct an investigation into medical records, and use the information to populate a database comparing all known patients with NSF verified in our laboratory. We use this information to guide medical, pharmaceutical, and government advisory personnel, and to guide further research into the cause of this mysterious disorder.
Our goal is to eradicate this dangerous (and sometimes deadly) condition, and to use the information to better understand fibrosis and wound healing in the hopes of impacting research on related conditions.
Specialized Terms: Alopecia; Cutaneous lymphoma; Pathology informatics; Nephrogenic fibrosing dermopathy; Nephrogenic systemic fibrosis
My primary research interest is in the mechanism of nephrogenic systemic fibrosis (formerly nephrogenic fibrosing dermopathy). This is an emerging fibrosing disorder seen in patients with renal disease. Compelling epidemiological, microanalytical, and animal studies have linked the disease to the use of gadolinium-based contrast agents (GBCA) used in MRI imaging. Current thinking suggests these renally-excreted compounds are ineffectively removed in the renally-impaired, leading to a novel toxicity reaction. The findings manifest themselves most dramatically in the skin and soft-tissue of affected patients by increased production of matrix elements (collagen, elastin, mucin). These excess components reduce the flexibility and compliance of skin, leading to decreased range of motion in affected limbs. Questions currently being examined include effects in non-cutaneous tissues and factors that may contribute to a propensity to develop NSF (genetic, medical cofactors, other toxicities, etc).
Yale is home to the International NSF Registry, housing clinical records and pathology specimens from over 330 patients with NSF. This registry serves as a source of basic information for investigations into the clinical and histological aspects of NSF.
Yale is also the home base for a core of interested clinical and basic science researchers actively engaged in understanding all aspects of this unusual disease. Periodically, this team hosts the Yale Fibrosis Symposium.
Besides NSF, I am interested in cutaneous lymphomas and alopecias, and the appropriate use of histopathology in diagnosing these disorders.
My primary and preferred instructional tool is the networked computer. I have been involved with web-based pathology education for some time. My largest project Pathmax, was one of the first web-based resource for pathology professionals on the internet. Beginning in 1997, this site ran continuously until 2018. I am also one of the cofounders of the Dermatopathology Master Class Series, a web-based streaming lecture series on topics of relevance in dermatopathology. In recent years I have been the chairman of the Virtual Slide Library of the American Society of Dermatopathology where I manage 50 volunteers interested in optimizing and vetting over 10,000 scanned dermatopathology whole slide images.
Alopecia; Dermatology; Fibrosis; Medical Informatics; Pathology; Nephrogenic Fibrosing Dermopathy