Acute localized scleroderma (morphea) can present as severe generalized oedema with rapid weight gain and oliguria. The putative mechanism is increased capillary permeability.
CASE HISTORYA woman of 67 was referred to the renal service for investigation of severe and increasing peripheral oedema which had not responded to treatment with diuretics. Her weight had risen by 12 kg in a few weeks. On close questioning she indicated that the skin of her swollen legs had become abnormal in texture, 'hard', and she had also noticed hardening of the skin of the forearms and lower abdomen. 7 years previously seronegative rheumatoid arthritis had been diagnosed, mainly affecting her hands; this had responded to weekly methotrexate, which she was still taking.There was sacral and pretibial oedema, with abdominal swelling; the lungs were normal and jugular venous pressure was not raised. She had striking dermatographia, acrosclerosis and hardening of the skin of both legs. Dipstick testing of urine revealed neither proteinuria nor haematuria. On admission, plasma sodium was 138 mmol/L, potassium 3.9 mmol/L, albumin 28 g/L, urea 4.7 mmol/L and creatinine 76 mmol/L. C-reactive protein was 70 mg/L. Haemoglobin was 11.9 g/L with normal white cell count and differential; platelet count was 454Â10 9 /L. Results of the following were all normal: plasma glucose, calcium, phosphate, bilirubin, alanine aminotransferase, alkaline phosphatase, immunoglobulins and protein electrophoresis, and complement C3 and C4; clotting tests; thyroid function tests; bone marrow biopsy; and abdominal CT scan (apart from generalized oedema). An echocardiogram showed normal left ventricular function but there was some dilatation of the right ventricle with moderate tricuspid regurgitation. Initial urine volume was only 500 mL/day, though creatinine clearance was normal. Albuminuria remained undetectable, but she had tubular proteinuria; 24-hour urinary sodium excretion was very low (12 mmol/24 h) and remained so for several weeks; urine osmolality was 982 mosm/kg, with plasma osmolality 294 mosm/kg. Colloid oncotic pressure was slightly reduced at 19 mmHg (normal range 20-23). After admission she gained a further 20 kg (about 1 kg/day), despite fluid restriction. Further investigations showed serum aldosterone normal, renin about twice the upper limit of normal, rheumatoid factor negative, antinuclear antibody (ANA) weakly positive at 1:20, anti Ro, La, Sm, RNP, Jo-1 and SCL070 all negative. Urinary cortisol was normal.A diagnosis of morphea was suggested and was confirmed by skin biopsies. She was treated first with methylprednisolone, then with oral steroids and a trial of penicillamine. The skin disorder stabilized and the oedema subsided over a few months. Renal function, which did not change during the hospital admission, remained stable during 2 years of follow up with plasma creatinine around 90 mmol/L and an isotopic glomerular filtration rate of 60 mL/min/m 2 . She has required treatment for hypertension, currently well controlled on two drugs....