What are the other Names for this Condition? (Also known as/Synonyms)

• NFD (Nephrogenic Fibrosing Dermopathy)

What is Nephrogenic Fibrosing Dermopathy? (Definition/Background Information)

• Nephrogenic Fibrosing Dermopathy (NFD) is a rare condition, first reported in 1997, in which there is a sudden onset of nodular or plaque-like skin lesions. The condition can resemble scleroderma in appearance
• The condition is strongly linked to exposure to certain types of gadolinium contrast material during magnetic resonance imaging (MRI) studies. Nearly all cases have developed in individuals undergoing dialysis for kidney failure
• Nephrogenic Fibrosing Dermopathy mostly affects adults - young, middle-aged, and old. The skin nodules or plaques mostly occurs on the legs and sometimes on the hands
• In addition to skin fibrosis, there may be fibrosis of other major organs of the body such as the heart, lungs, or liver. This can result in disruption of their function causing severe complications
• It is difficult to treat Nephrogenic Fibrosing Dermopathy; though in some patients the use of monoclonal antibodies and other interventions has been found to be beneficial
• The prognosis of this chronic disorder is typically poor in spite of appropriate treatment, especially if associated with systemic fibrosis, when many important organs are affected

Who gets Nephrogenic Fibrosing Dermopathy? (Age and Sex Distribution)

• Nephrogenic Fibrosing Dermopathy is a very infrequently occurring condition that can be observed in young, middle-aged, and older adults; in the 30-80 years age category
• Both male and female genders are affected
• Individuals of all racial and ethnic groups may be affected

It is seen in those who have recently been exposed to certain types of gadolinium given for MRI testing. It is most commonly associated with Group I (linear) gadolinium agents. The use of Group I gadolinium contrast agents has more recently been reduced around the world.

What are the Risk Factors for Nephrogenic Fibrosing Dermopathy? (Predisposing Factors)

Following are the risk factors for Nephrogenic Fibrosing Dermopathy:

• Exposure to gadolinium, which is used as a contrast material for magnetic resonance imaging (MRI) studies
• Kidney failure necessitating dialysis
• Individuals with hepatitis C virus (HCV) infection
• Those undergoing systemic immunosuppressive therapy for various causes

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Nephrogenic Fibrosing Dermopathy? (Etiology)

The cause of development of Nephrogenic Fibrosing Dermopathy is unknown.

• Most of the cases occur in patients having kidney failure and receiving dialysis therapy
• NFD is also associated with the contrast material gadolinium, which is used for imaging studies in individuals with kidney conditions
• It can also occur in cases, where there is acute kidney failure, but the patient has not undergone dialysis yet

What are the Signs and Symptoms of Nephrogenic Fibrosing Dermopathy?

The signs and symptoms of Nephrogenic Fibrosing Dermopathy may include the following:

• The appearance of skin lesions that are like nodules or plaques; these lesions may appear suddenly (sudden onset). They can measure up to 20 cm in size
• The lesions are painful, itchy, and there can be tingling sensation of the affected area
• The skin can have a woody consistency or texture
• Most commonly the lower legs are involved, while sometimes the arms and hands may be affected. However, usually the face is not involved
• Yellowish plaques of the sclera (whites  of the eye)
• The fibrosis can not only affect the skin, but also the internal organs. This is called Nephrogenic Systemic Fibrosis (NSF)

How is Nephrogenic Fibrosing Dermopathy Diagnosed?

Diagnostic tests that are performed for Nephrogenic Fibrosing Dermopathy (NFD) may include:

• A thorough physical examination and evaluation of a complete medical history
• Dermoscopy: Dermoscopy is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
• Blood tests, to rule out other causes such as infections
• Potassium hydroxide (KOH) test: The KOH prep involves microscopic observation of skin scrapings for any fungal infections
• Allergy skin test
• Skin biopsy: A biopsy of the lesion is usually performed to confirm the diagnosis. A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. In cases of suspected NFD and NSF, the skin biopsy must include the deep dermis and into the subcutaneous fat, as the fibrosis extends down into the subcutis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Nephrogenic Fibrosing Dermopathy?

The complications of Nephrogenic Fibrosing Dermopathy (NFD) may include:

• It can severely affect the function of the involved organ such as the heart, lung, liver, and brain
• Fibrosis of the skin across joints can cause joint contractures with limb immobility
• Secondary skin infections
• Emotional and psychological stress due to cosmetic issues arising from the wood-like skin
• NFD is a chronic condition that can be fatal due to the involvement of several vital organs

How is Nephrogenic Fibrosing Dermopathy Treated?

• Nephrogenic Fibrosing Dermopathy is generally difficult to treat
• Newer monoclonal antibody treatments have been found to be helpful in some individuals
• Some treatment measures to improve comfort may include:
  • Maintain cleanliness and body hygiene
  • Using moisturizers or other lotions to prevent the skin from drying
  • Use of topical steroidal creams and lotions
  • Antibiotics may be used to treat infections
• Undertaking treatment of any underlying disorders or conditions

How can Nephrogenic Fibrosing Dermopathy be Prevented?

Currently, it is not possible to prevent Nephrogenic Fibrosing Dermopathy (NFD). However, the following factors may be considered to avoid NFD:

• The use of gadolinium agents more rapidly excreted despite renal dysfunction has lowered the rate of NFD/NSF considerably. Group II (macrocyclic) gadolinium agents are better excreted than Group I (linear) agents
• Adequately treat kidney disease and kidney failure
• Careful calculation of the cumulative  dose of gadolinium injected on a weight basis when multiple studies are being considered
• Closely monitor individuals receiving immunosuppressants for various reasons
• Minimize the use of MRI studies with Group I (linear) gadolinium contrast agents in ‘at-risk’ patients[JF1] with impaired kidney function on dialysis
• It important to note that the newer Group II macrocyclic gadolinium agents are safe even with chronic kidney dysfunction patients on dialysis
• There is a proposed link of NFD/NSF with liver disease, particularly hepatitis C, which has not yet been determined

What is the Prognosis of Nephrogenic Fibrosing Dermopathy? (Outcomes/Resolutions)

• The prognosis of Nephrogenic Fibrosing Dermopathy is generally poor despite suitable treatment
• The prognosis also depends on the severity of the underlying condition and the involvement of various internal organs

Additional and Relevant Useful Information for Nephrogenic Fibrosing Dermopathy:

End-stage kidney disease (ESKD) is a condition defined by near-complete or complete failure of the kidneys to excrete waste products and excess water from the body.