Shawn Cowper, MD
- Associate Professor of Dermatology and Pathology
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. Annually, this team hosts the Yale NSF Symposium, the largest gathering of its type on the topic of NSF in the world. The upcoming 4th annual symposium will take place at the New York Academy of Sciences on May 14/15, 2010.
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 is Pathmax, a web-based resource for pathology professionals currently accommodating 60,000 unique visitors per year from over 80 countries. 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.
- MD, Michigan State University, 1990
- An Atlas of Hair Pathology with Clinical Correlations, 2d Ed. (Book): Sperling L, Cowper SE, Knopp EA. Informs Healthcare, 2012
- Girardi M, Kay J, Elston DM, Leboit PE, Abu-Alfa A, Cowper SE. Nephrogenic systemic fibrosis: clinicopathological definition and workup recommendations. Journal Of The American Academy Of Dermatology 2011, 65:1095-1106.e7.
- Cowper SE. Gadolinium - is it to blame? Journal Of Cutaneous Pathology 2008, 35:520-2.
- Knopp EA, Cowper SE. Nephrogenic systemic fibrosis: early recognition and treatment. Seminars In Dialysis 2008, 21:123-8.
- Cowper SE, Rabach M, Girardi M. Clinical and histological findings in nephrogenic systemic fibrosis. European Journal Of Radiology 2008, 66:191-9.
- Moreno-Romero JA, Segura S, Mascaró JM, Cowper SE, Julià M, Poch E, Botey A, Herrero C. Nephrogenic systemic fibrosis: a case series suggesting gadolinium as a possible aetiological factor. The British Journal Of Dermatology 2007, 157:783-7.
- Cowper SE, Kuo PH, Bucala R. Nephrogenic systemic fibrosis and gadolinium exposure: association and lessons for idiopathic fibrosing disorders. Arthritis And Rheumatism 2007, 56:3173-5.
- High WA, Ayers RA, Cowper SE. Gadolinium is quantifiable within the tissue of patients with nephrogenic systemic fibrosis. Journal Of The American Academy Of Dermatology 2007, 56:710-2.
- Morcos SM, Girardi M, Subtil A, Wilson LD, Cowper SE. Mycosis fungoides exhibiting features of a dermatofibroma: a case report and review of the literature. Journal Of Cutaneous Pathology 2012, 39:40-6.
- Igreja AC, Mesquita Kde C, Cowper SE, Costa IM. Nephrogenic systemic fibrosis: concepts and perspectives. Anais Brasileiros De Dermatologia 2012, 87:597-607.
- Nardone B, Saddleton E, Laumann AE, Edwards BJ, Raisch DW, McKoy JM, Belknap SM, Bull C, Haryani A, Cowper SE, Abu-Alfa AK, Miller FH, Godinez-Puig V, Dharnidharka VR, West DP. Pediatric nephrogenic systemic fibrosis is rarely reported: a RADAR report. Pediatric Radiology 2014, 44:173-80.
- Thomson LK, Thomson PC, Kingsmore DB, Blessing K, Daly CD, Cowper SE, Roditi GH. Diagnosing nephrogenic systemic fibrosis in the post-FDA restriction era. Journal Of Magnetic Resonance Imaging : JMRI 2015, 41:1268-71.
- Edwards BJ, Laumann AE, Nardone B, Miller FH, Restaino J, Raisch DW, McKoy JM, Hammel JA, Bhatt K, Bauer K, Samaras AT, Fisher MJ, Bull C, Saddleton E, Belknap SM, Thomsen HS, Kanal E, Cowper SE, Abu Alfa AK, West DP. Advancing pharmacovigilance through academic-legal collaboration: the case of gadolinium-based contrast agents and nephrogenic systemic fibrosis-a Research on Adverse Drug Events and Reports (RADAR) report. The British Journal Of Radiology 2014, 87:20140307.