Heck's disease: diagnosis and susceptibility.

Focal epithelial hyperplasia, or Heck's disease, is an uncommon proliferation of oral mucosa that presents primarily in Native Central and South American populations. It presents as asymptomatic papules or nodules on the oral mucosa, gingiva, tongue, and lips. In the majority of cases, human papilloma virus 13 or 32 is detected. Factors that determine disease susceptibility are unclear, but genetics, and having the human lymphocytic antigen-DR4 (DRB1*0404) allele in particular, are thought to play a major role in disease vulnerability. We report another case of focal epithelial hyperplasia, hypothesize on disease susceptibility, and review the current understanding of this uncommon disorder.

Nephrogenic systemic fibrosis: is gadolinium the missing piece to the puzzle?

New medical disorders arise infrequently, but nephrogenic systemic fibrosis (NSF) is one such entity. It exclusively affects patients with renal failure, resulting in debilitating progressive fibrosis of the skin and systemic organs. Although much work has been done elucidating the histopathologic changes, a trigger has not been detected. Recently, case reports have implicated gadolinium (Gd) contrast agents as a potential etiology, prompting a health advisory from the US Food and Drug Administration (FDA) in June 2006. We discuss the literature regarding the effects of Gd on tissue and its potential relationship to the known histopathologic characteristics of NSF.

Nephrogenic systemic fibrosis: risk factors and incidence estimation.

PURPOSE: To retrospectively review data in 13 patients with biopsy-confirmed nephrogenic systemic fibrosis (NSF), assess the associated risk factors, and report the incidence of NSF at the authors' institution. MATERIALS AND METHODS: This HIPAA-compliant study had institutional review board approval; informed consent was waived. Statistical analysis was performed for all available clinical and laboratory data in patients with biopsy-confirmed NSF. The data from the patients with NSF were compared with data from a control population of patients with renal insufficiency but who did not develop NSF. RESULTS: There were eight male and five female patients, aged 17-69 years, with a diagnosis of NSF. Within 6 months of diagnosis, all 13 patients had been exposed to gadodiamide and one had been exposed to gadobenate dimeglumine in addition to gadodiamide. At the time of contrast material-enhanced magnetic resonance (MR) imaging, all 13 patients had renal insufficiency (estimated glomerular filtration rate [eGFR] < 60 mL/min/1.73 m(2)) and were hospitalized for a proinflammatory event (major surgery, infection, or vascular event). The group with NSF had significantly decreased eGFR (P = .01), more proinflammatory events (P < .001), and more contrast-enhanced MR examinations per patient (P = .002) than did the control group. CONCLUSION: A combination of factors, including altered kidney function, inflammatory burden, and exposure to gadolinium-based contrast agents may all play a role in development of NSF. Alternative imaging should be considered in patients with these factors. If use of a gadolinium-based agent is clinically indicated, the referring physician and patient should be informed of the potential risk of developing NSF.