SUMMARY A 57 year old man with nail-patella syndrome (NPS) and associated renal disease is described who developed an inflammatory polyarthropathy and polyarteritis-like vasculitis. Vasculitis and serum complement abnormalities have not previously been reported in NPS. NPS is a rare autosomal dominant connective tissue disorder affecting both mesenchymal and ectodermal tissue. The condition is reviewed with particular reference to its renal pathology, including the distinctive electron microscopic (EM) finding of collagen deposition in the glomerular basement membrane (GBM). The possibility of the underlying collagen abnormality acting as a trigger for immune-inflammatory changes is discussed.Key words: collagen, basement membrane, polyarteritis.Case report A 57 year old white man was hospitalised in November 1985 for investigation of symmetrical polyarthritis and recent onset paraesthesia and petechial skin rash of the legs (two weeks). We noted typical clinical features of NPS: positive family history, hypoplastic thumbnails and patellae, and severe knee and elbow arthropathy bilaterally (15 years). There was a six month history of significant morning stiffness (four hours) in association with bilateral symmetrical pain and swelling of elbows, wrists, and metacarpophalangeal (MCP) joints, occasionally relieved by non-steroidal drug therapy. The past history was also notable for chronic renal failure, hypertensive heart disease, and atrial fibrillation (10 years). A right nephrectomy in 1982, at which time the creatinine clearance was 70 ml/min, showed a small end stage kidney with an embolised right renal artery. Routine histology showed generalised glomerulosclerosis and cortical atrophy.

Tumoral calcinosis (TC) is an uncommon disorder characterized by large calcific soft tissue deposits, usually adjacent to large joints. In the past, the etiology was unclear, but recently, TC has been considered to be an inherited metabolic disorder, characterized by elevated serum phosphorus and 1,25-dihydroxyvitamin D levels, normocalcemia, and calcific deposits. However, these features have been shown to vary widely, with formes frustes of TC now being recognized. We report an unusual case of TC, which presented as an adhesive capsulitis, in an elderly North American black woman. The patient's nationality, late age of presentation, aad multiple sites of involvement are uncommon features of TC. The clinicopathologic features and possible etiologies of this rare condition are briefly reviewed.Case report. The patient, a 76-year-old North American black woman, presented with a 3-month history of left shoulder and arm pain of insidious onset. No previous shoulder or neck trauma was reported. The pain involved the entire shoulder and upper arm circumference, was constant, and caused increased restriction of all shoulder movement to the point that the patient required assistance in dressing and bathing. Nonsteroidal antiinflammatory drug therapy, prescribed by a family physician who had diag- Physical examination revealed an obese, afebrile,edentulous female with a blood pressure of 160/80 mm Hg, normal pulses, and no evidence of systemic disease. Of note, there was no lymphadenopathy, intrathoracic disease, abdominal visceromegaly , generalized myopathy, or neuropathy.Examination of the left shoulder revealed features of an adhesive capsulitis, with minimal mobility in all ranges of motion tested. Deep palpation around the left shoulder and left thigh, circumferentially, elicited tenderness. There were no abnormalities of the other joints of the arms, nor was there evidence of bicipital tendinitis or carpal tunnel syndrome. Mobility of both hip joints was normal, and straight-leg raising was not limited. The trochanteric bursae were not tender. The provisional clinical diagnosis was adhesive capsulitis of the left shoulder and nonspecific left thigh pain.Laboratory investigations revealed a normal hemoglobin level (13.7 gddl), white blood cell count (5,000/mm3), and platelet count (216,000/mm3). The Westergren erythrocyte sedimentation rate was normal (35 mdhour). Calcium and inorganic phosphorus levels were normal (9.4 mg/dl and 3.8 mg/dl, respectively), thereby excluding overt parathyroid disease