Previous name: scleromyxedema-like illness of renal disease
Clinical features:
(1) The patient has end-stage renal disease, often is on dialysis.
(2) The patient develops a painful cutaneous fibrosis that affects the limbs and trunk but spares the face.
(3) The patient develops flexion joint contractures with limited range of motion.
(4) Fibrosis may extend deep to involve the fascia and muscles of the extremities.
(5) Soft tissue calcifications may be present.
Some cases have occurred in patients with renal insufficiency who were given a gadolinium-based contrast agent for an MRI.
Skin manifestations:
(1) induration and thickening of the skin
(2) plaques
(3) brawny hyperpigmentation
(4) papules
(5) subcutaneous nodules
Laboratory findings:
(1) elevated ESR
(2) elevated C-reactive protein
(3) no evidence of a paraproteinemia
A skin biopsy of a developed lesion will show:
(1) thick collagen bundles in the dermis that may also extend into the subcutaneous fat
(2) a proliferation of fibroblasts which are CD-34 positive
(3) increased mucin
Differential diagnosis:
(1) scleroderma (skin fibrosis should precede renal dysfunction)
(2) scleromyxedema (associated with a paraproteinemia)
(3) eosinophilic fasciitis
