Shawn Edwin Cowper MD

Associate Professor of Dermatology and of Pathology

Research Interests

Alopecia; Cutaneous lymphoma; Pathology informatics; Nephrogenic fibrosing dermopathy; Nephrogenic systemic fibrosis

Research Summary

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.

Extensive Research Description

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 effected 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.

Selected Publications

  • An Atlas of Hair Pathology with Clinical Correlations, 2d Ed. (Book): Sperling L, Cowper SE, Knopp EA. Informa Healthcare, 2012
  • Girardi M, Kay J, Elston DM, Leboit PE, Abu-Alfa A, Cowper SE. Nephrogenic systemic fibrosis: Clinicopathological definition and workup recommendations. J Am Acad Dermatol. 2011 Jul 1. [Epub ahead of print]
  • Cowper, SE. Gadolinium: Is it to blame? Jour Cut Pathol 35:520-2. 2008
  • Knopp E, Cowper SE. Nephrogenic Systemic Fibrosis: Early Recognition and Treatment. Semin Dial. 2008 Mar-Apr;21(2):123-8. Epub 2008 Jan 23.
  • Cowper SE, Rabach M, Girardi M. Clinical and Histological Findings in Nephrogenic Systemic Fibrosis. Eur J Radiol. 2008 May;66(2):191-9. Epub 2008 Mar 5.
  • Moreno-Romero JA, Tigell SS, Mascaro JM, Mateu CH, Cowper SE. Nephrogenic systemic fibrosis: A case series with gadolinium as a possible etiologic factor. Br J Dermatol 157(4):783-7. Oct 2007 Epub 2007 Jul 11
  • Cowper SE, Kuo PH, Bucala R. Nephrogenic Systemic Fibrosis and Gadolinium Exposure: Association and Lessons for Idiopathic Fibrosing Disorders. (Invited editorial) Arth and Rheum 56(10): 3173-5. Sep 2007
  • High WA, Ayers RA, Cowper SE. Gadolinium is quantifiable within tissue of patients with nephrogenic systemic fibrosis. J Amer Acad Dermatol 56(4): 710-2. April 2007

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