Alopecia; Dermatology; Fibrosis; Medical Informatics; Pathology; Nephrogenic Fibrosing Dermopathy
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
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 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.
- An Atlas of Hair Pathology with Clinical Correlations, 2d Ed. (Book): Sperling L, Cowper SE, Knopp EA. Informs Healthcare, 2012
Nephrogenic systemic fibrosis: Clinicopathological definition and workup recommendations.
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]
Gadolinium: Is it to blame?
Cowper, SE. Gadolinium: Is it to blame? Jour Cut Pathol 35:520-2. 2008
Nephrogenic Systemic Fibrosis: Early Recognition and Treatment.
Knopp E, Cowper SE. Nephrogenic Systemic Fibrosis: Early Recognition and Treatment. Semin Dial. 2008 Mar-Apr;21(2):123-8. Epub 2008 Jan 23.
Clinical and Histological Findings in Nephrogenic Systemic Fibrosis.
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.
Nephrogenic systemic fibrosis: A case series with gadolinium as a possible etiologic factor.
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
Nephrogenic Systemic Fibrosis and Gadolinium Exposure: Association and Lessons for Idiopathic Fibrosing Disorders.
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
Gadolinium is quantifiable within tissue of patients with nephrogenic systemic fibrosis.
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
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.
J Cutan Pathol. 2012 Jan;39(1):40-6. doi: 10.1111/j.1600-0560.2011.01804.x. Epub 2011 Nov 8
Igreja AC, Mesquita Kde C, Cowper SE, Costa IM. Nephrogenic systemic fibrosis: concepts and perspectives.
An Bras Dermatol. 2012 Jul-Aug;87(4):597-607. Review.
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.
Pediatr Radiol. 2014 Feb;44(2):173-80. doi: 10.1007/s00247-013-2795-x. Epub 2013 Sep 21.
Thomson LK, Thomson PC, Kingsmore DB, Blessing K, Daly CD, Cowper SE, Roditi GH. Diagnosing nephrogenic systemic fibrosis in the post-FDA restriction era.
J Magn Reson Imaging. 2015 May;41(5):1268-71. doi: 10.1002/jmri.24664. Epub 2014 Jun 5.
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.
Br J Radiol. 2014 Oct;87(1042):20140307. doi: 10.1259/bjr.20140307.
Full List of PubMed Publications
- Panse G, Cowper SE, Leffell DJ, Pulitzer M, Ko CJ: Well-differentiated neuroendocrine tumors in skin: Terminology and diagnostic utility of cytokeratin 5/6 and p63. J Cutan Pathol. 2017 Jun. PMID: 28417484
- Huang J, Cowper S, Moss J, Girardi M: Case experience of 308-nm excimer laser therapy compatibility with PUVA and oral bexarotene for the treatment of cutaneous lesions in mycosis fungoides. J Drugs Dermatol. 2013 Apr. PMID: 23652902
- 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. J Cutan Pathol. 2012 Jan; 2011 Nov 8. PMID: 22059953
- 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 Dec; 2011 Jul 2. PMID: 21724294
- Braverman IM, Cowper S: Nephrogenic systemic fibrosis. F1000 Med Rep. 2010 Nov 24; 2010 Nov 24. PMID: 21283650
- Spock CR, Masood Q, Cowper SE, Narayan D: Fibrosis of the hand secondary to jet propellant exposure: a new clinical syndrome. Plast Reconstr Surg. 2009 Nov. PMID: 20009810
- Cowper SE, Rabach M, Girardi M: Clinical and histological findings in nephrogenic systemic fibrosis. Eur J Radiol. 2008 May; 2008 Mar 5. PMID: 18325705
- Cowper SE: Gadolinium - is it to blame? J Cutan Pathol. 2008 May. PMID: 18399814
- Knopp EA, Cowper SE: Nephrogenic systemic fibrosis: early recognition and treatment. Semin Dial. 2008 Mar-Apr; 2008 Jan 23. PMID: 18226008
- Cowper SE: Nephrogenic systemic fibrosis: an overview. J Am Coll Radiol. 2008 Jan. PMID: 18180005
- Cowper SE: Nephrogenic systemic fibrosis: a review and exploration of the role of gadolinium. Adv Dermatol. 2007. PMID: 18159899
- Galan A, Cowper SE, Bucala R: Nephrogenic systemic fibrosis (nephrogenic fibrosing dermopathy). Curr Opin Rheumatol. 2006 Nov. PMID: 17053507
- DeHoratius DM, Cowper SE: Nephrogenic systemic fibrosis: an emerging threat among renal patients. Semin Dial. 2006 May-Jun. PMID: 16689966
- Cowper SE, Boyer PJ: Nephrogenic systemic fibrosis: an update. Curr Rheumatol Rep. 2006 Apr. PMID: 16569375
- Quan TE, Cowper SE, Bucala R: The role of circulating fibrocytes in fibrosis. Curr Rheumatol Rep. 2006 Apr. PMID: 16569374
- McNiff JM, Cowper SE, Lazova R, Subtil A, Glusac EJ: CD56 staining in Merkel cell carcinoma and natural killer-cell lymphoma: magic bullet, diagnostic pitfall, or both? J Cutan Pathol. 2005 Sep. PMID: 16115052
- McNiff JM, Subtil A, Cowper SE, Lazova R, Glusac EJ: Cellular digital fibromas: distinctive CD34-positive lesions that may mimic dermatofibrosarcoma protuberans. J Cutan Pathol. 2005 Jul. PMID: 15953374
- Quan TE, Cowper S, Wu SP, Bockenstedt LK, Bucala R: Circulating fibrocytes: collagen-secreting cells of the peripheral blood. Int J Biochem Cell Biol. 2004 Apr. PMID: 15010326
- Cowper SE: Nephrogenic fibrosing dermopathy: the first 6 years. Curr Opin Rheumatol. 2003 Nov. PMID: 14569211
- Cowper SE, Rosenberg AS, Morgan MB: An investigation of apoptosis in androgenetic alopecia. Am J Dermatopathol. 2002 Jun. PMID: 12140435