Nephrogenic fibrosing dermopathy in a renal transplant recipient with tubulointerstitial nephritis and uveitis.
We report a patient with nephrogenic fibrosing dermopathy. He had chronic renal failure with arthritis, uveitis and histologically severe tubulointerstitial nephritis for which he received a renal transplant from a family relative. After an episode of acute renal failure with the transplant he developed painful, erythematous, firm papules and plaques with geographic borders on the legs, anterior thorax and elbow. A skin biopsy revealed increased fibroblast and collagen fiber content of the dermis and subcutaneous septae. Mucin deposition, sparse smooth-muscle-actin-positive cells and an increased number of CD34-positive cells in the deep dermis were found. After several weeks of hemodialysis, the lesions changed from an inflammatory to a purely sclerotic phase. The fibrocyte, a recently described circulating cell type, that is deposited in scar tissue may be involved in the pathogenesis of this novel pseudosclerodermatous skin disorder.
Hauser
Conrad
C
Division of Immunology and Allergy, Department of Internal Medicine, Geneva University Hospital, Geneva, Switzerland. conrad.hauser@medecine.unige.ch
Kaya
Gürkan
G
Chizzolini
Carlo
C
D003937:Diagnosis, Differential; D004550:Elbow; D005355:Fibrosis / Q000150:complications / Q000175:diagnosis / Q000473:pathology; D006801:Humans; D016030:Kidney Transplantation; D007866:Leg; D008297:Male; D008875:Middle Aged; D009395:Nephritis, Interstitial / Q000150:complications*; D012871:Skin Diseases / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D013909:Thorax; D014605:Uveitis / Q000150:complications*