We searched the autopsy records of the Department of Pathology at the Massachusetts General Hospital from 2004-2008 for the diagnostic terms nephrogenic systemic fibrosis and nephrogenic fibrosing dermopathy
3) A reporting register has been set up on the web: International Center for Nephrogenic Fibrosing Dermopathy
Research (ICNFDR) www.
Nephrogenic fibrosing dermopathy
(NFD) is marked by areas of tight, rigid skin and may progress to nephrogenic systemic fibrosis (NSF), which is associated with scarring of internal organs.
A small study presented at the annual meeting of the American Association of Clinical Endocrinologists found that 31 of 39 patients with acropachy (79%) and 81 of 102 patients with dermopathy
(79%) had a history of smoking.
There was, however, no evidence of any focal neurologic deficit, and there was no evidence of ophthalmopathy or dermopathy
characteristic of Graves' disease.
The syndrome commonly consisted of weight loss, weakness, thyromegaly, hepatomegaly, gross porphyrinuria, hypertricosis, and photosensitive dermopathy
Contrast-free imaging is particularly important because gadolinium, the most common contrast agent used for magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) exams, has been directly linked to a sometimes fatal disease that occurs in patients with renal insufficiency, called Nephrogenic Systemic Fibrosis (NSF) or Nephrogenic Fibrosing Dermopathy
There were no specific signs of Graves' disease like ophthalmopathy and dermopathy
The disease was designated nephrogenic fibrosing dermopathy
These contrast agents have been linked to nephrogenic systemic fibrosis (NSF) and nephrogenic fibrosing dermopathy
Nephrogenic systemic fibrosis, previously known as nephrogenic fibrosing dermopathy
, is a newly recognized systemic fibrosing disorder primarily affecting patients with chronic renal failure.
Worldwide, there have been only 170 cases of nephrogenic fibrosing dermopathy
(NFD) reported since it was first described in 1997.
They include acanthosis nigricans and NLD, both seen in this patient, as well as bullous diabeticorum, diabetic dermopathy
, eruptive xanthomas, generalized granuloma annulare, infections (yeast or bacterial), neuropathic foot ulcers, and scleredema.
11) In one series of 150 patients with Graves' dermopathy
reported from the Mayo Clinic, only 1 patient did not have accompanying ophthalmopathy.
Using non-contrast techniques eliminates the risks associated with gadolinium-based contrast agents such as the rare and sometimes fatal skin disease Nephrogenic Systemic Fibrosis or Nephrogenic Fibrosing Dermopathy