Scleromyxedema-like lesions of patients in renal failure contain hyaluronan: a possible pathophysiological mechanism.

BACKGROUND

Patients with renal failure have been identified recently, some on dialysis, others with renal transplants, who have scleromyxedema-like skin changes. These lesions are characterized grossly by extensive thickening of skin, brawny pigmentation, papules, and subcutaneous nodules. Mucinous deposits are observed histologically that resemble those in scleromyxedema.

METHODS

Biopsies of these lesions were stained with a biotinylated hyaluronan (HA)-binding protein coupled to an avidin-peroxidase reaction.

RESULTS

These lesions are associated with marked deposition of HA in the papillary dermis.

CONCLUSIONS

HA turnover is cleared rapidly in the circulation by both liver and kidney. Evidence suggests that high molecular size HA chains, which are anti-inflammatory, antiangiogenic, and immuno-suppressive are cleared by the liver. By contrast, intermediate-size fragments, which are highly angiogenic, inflammatory, and a stimulus for fibrous deposition, are cleared by the kidney. The accumulation of such fragments in renal failure can account for HA deposition in the dermis and may be a mechanism for the nephrogenic fibrosing dermopathy that can accompany these lesions.