CLINICAL Our case involves a 36-year-old female patient with systemic lupus erythematosus and end-stage renal disease requiring hemodialysis. She presented in May 2002 with large areas of painful skin involving her arms and legs, developing during the prior 6 months. The lesions were composed of elevated pale yellow plaques with sharp margination. Some lesions showed stippling while others had a peau d’orange change. The patient required narcotic analgesics because the involved skin areas were very tender.
HISTOLOGY Biopsy showed changes suggestive of scleromyxedema. A colloidal iron stain was positive for mucin. A CD34 stain was positive. The patient had neither a monoclonal gammopathy nor paraproteinemia.
IMPORTANT FEATURE: REASON FOR PRESENTATION In 2000 Dr. Philip LeBoit and others (1) described 15 patients with severe renal disease who had developed a scleroderma-like condition on the extremities. They later termed this disease nephrogenic fibrosing dermopathy (NFD). This is the 50th case report of this newly-described cutaneous disease occurring in patients with severe renal disease. All but two of the earlier patients needed hemo- or peritoneal dialysis. In one patient the cutaneous disease cleared when dialysis was no longer required. NFD resembles scleromyxedema and the eosinophilia-myalgia syndrome caused by contaminated tryptophan. It also has features similar to Spanish oil syndrome related to tainted cooking oil. Like these latter two disorders, NFD may be the result of a toxin or contaminant related to dialysis. The cause is under investigation by CDC (3). More cases should be anticipated.

REFERENCES

1. Cowper S E, Robin HS, Steinberg S M, et al. Scleromyxedema-like cutaneousdiseases in renal dialysis patients. (Research letter.) Lancet 2000(Sep16); 356:1000-1001.

2. Cowper S E, Su LD, Bhawan J, et al. Nephrogenic fibrosing dermopathy. Am J Dermatopathol 2001(Oct); 23(5):383-393.

3. Center for Disease Control and Prevention. Fibrosing skin condition among patients with renal disease — United States and Europe, 1997-2002. MMWR 2002; 51(2):25-26.