Nephrogenic Systemic Fibrosis

Key Points

Most patients have been given the MRI contrast agent, gadolinium, prior to onset of the condition
Rare condition which typically affects the skin, but may affect internal organs as well
Exact cause is unknown, but appears in relation to kidney disease
Cutaneous symptoms consist of swelling and tightening of the skin, which may become thickened and hard and be accompanied by various other symptoms

Nephrogenic fibrosing dermopathy is a rare condition which generally affects the skin, but may involve internal organs in some cases, and is known as nephrogenic systemic fibrosis. The condition typically consists of swelling and tightening of the skin, which becomes hard and thick. The skin develops a woodlike texture, and bumps or lumps may form on the affected area or around the eyes. Additionally, skin may be shiny, have brown pigmentation, joint movement may be hindered, and affected persons may experience muscle contractures and weakness. In a small percentage of affected persons, the condition is aggressive, and causes permanent damage very quickly.

Nephrogenic fibrosing dermopathy appears in relation to any stage of kidney disease, though its exact cause and why kidney disease triggers the condition is unknown. It is thought to be triggered by gadolinium, an MRI contrast agent. The condition displays no preference for any ethnic, age or gender group.

Differential Diagnosis (Other conditions with similar appearance)Scleromyxoedema
Scleredema
Eosinophilic fasciitis
Localized scleroderma
Systemic sclerosis

Diagnosis
Key Points
Initial diagnosis based on appearance of the affected area
Deep skin biopsy will be performed to attempt to confirm diagnosis

Nephrogenic fibrosing dermopathy is initially diagnosed based on the characteristic appearance of the affected area. A deep skin biopsy will be performed to confirm the diagnosis.

Treatment
Condition is chronic, and there is no specific treatment
Several therapies are available, with varying effectiveness against the condition
*In very rare cases, the condition has been seen to resolve itself to varying degrees

Nephrogenic fibrosing dermopathy is typically chronic, and no specific treatment exists. There are several therapies which have been used to varying degrees of effectiveness, including photodynamic therapy, IV Immunoglobulin, extracorporeal photopheresis, Systemic steroids, UV phototherapies, intralesional interferon, calcipotriol in conjunction with powerful topical steroids, plasmapheresis, sodium thiosulfate, and pentoxifylline. Physical therapy has been shown to assist in preventing loss of mobility due to muscle weakness and decreased joint function. In very rare cases, the condition has been seen to resolve itself, to varying degrees.