CASE HISTORY

This patient was a male of age 20 who had been perfectly well until 7th Jul, 1953, without any premonitory symptoms and in particular without any preceding sore throat, he suddenly developed a rash over both elbows and the finger tips. The rash was irritating and consisted at first of whitish raised areas about 1mm in diameter. After a few days the rash became dusky red in colour and definitely purpuric. The inner aspect of both thighs and the feet were involved at this time. He was treated by local applications and radiotherapy with considerable temporary improvement. Transferred to a hospital for further investigations and treatment. Examination on admission: pale youth with sub-orbital oedema, and pitting oedema of the legs and sacrum. Heart -normal. BP 140/80. Lungs and abdomen - normal. There was a severe albuminuria with many granular casts and red cells in the centrifuged deposit. There was a purpuric rash over the arms, back, buttocks and feet. There were bullous lesions containing bloodstained fluid on the toes. The blood urea on admission was 238mg/100ml. Electrolytes: Na 120, K 4.5, Cl 85. HCO3 20m.eg.l. Serum albumen 1.9g/l. Globulin 3.7g/l. Cholesterol 215mg/100ml. Urine protein 7.5g/l - later 20g/l. A skin biopsy confirmed the diagnosis of Henoch-Schonlein Purpura. The rash cleared a few days after admission. The oedema steadily became worse and he developed effusions into both pleural sacs and ascites. The albuminuria became increased in amount and the urine consistently contained red cells. Despite further trails of cortisone and repeated blood transfusions the general condition steadily deteriorated. 18th Sept, 1953. Some hypertension was found for the first time, blood pressure being 145/100. There was now severe oliguria and he was treated by an intra-gastric Borst-Bull drip together with ion exchange resins by mouth in view of a hyperkalaemia of 9.2m.eg/l. This resulted in temporary improvement after a few days treatment. 1st Oct 1953. The urine output had now risen to over 1 litre per day and the serum potassium had fallen to 3.9m.eg/l. The blood pressure had further increased to a level of 170/125. ECG changes suggestive of pericarditis. 9th October 1953; he became oliguric once more and this time did not respond to therapy. 14th Oct 1953: he was extremely dyspnoeic and there were moist sounds throughout the lung fields with frothy sputum suggestive of severe pulmonary oedema. He died in the evening of this day just over three months from the commencement of his illness. At postmortem he was well nourished with gross anasarca. The only significant findings were in the kidneys.

PATHOLOGY

Kidney 135mmx100mmx45mm. The specimen consists of one half of a kidney. At autopsy, the kidneys weighed approximately double normal and this mounted half kidney is obviously enlarged. Externally the capsule has stripped cleanly leaving a smooth surface but there is a hint of fine red speckling due actually to haemorrhage into glomerular tufts. On the cut surface the general architecture of the kidney is very well preserved; the cortex is of normal thickness, the junction is clear. There is some loss of definition in the cortical pattern. There are tiny reddish spots of haemorrhage, as on the outer surface, but the appearances as a whole are not diagnostic though they are suggestive.