The Cheshire cat syndrome.

Case 1 B.W.P. (39175), a boy of 6, in December 1965 developed prolonged intermittent fever and 2 months later was admitted to another hospital with abdominal pain, lymphadenopathy and a morbilliform rash, later scaling. Skin oedema and hair fall followed. He was started on prednisone but by May 1956 generalized oedema was present: he had lost weight and had difficulty in walking. He was admitted to the M.R.C. Rheumatism Unit at Taplow on 6 July 1956 on ACTH gel injections with a tentative diagnosis of dermatomyositis. He was severely ill with fever to 105°F, wasted and dehydrated with vomiting and polyuria. The limbs showed no contractures but there was a pigmented reticular rash on the legs (Fig. 2). This was thought to be more in favour of a vascular disease such as polyarteritis than of dermatomyosities. There was a polymorph leucocytosis. There was proteinuria, 20 mg/100 ml. I.V. fluids and hydrocortisone corrected his dehydration and electrolyte depletion but he remained desperately ill and febrile, occasionally reaching a temperature recorded as 106-4°F. Towards the end of August' a urinary infection developed and a blood culture grew coagulase positive Staph. aureus for which he was given chloramphenicol followed by other antibiotics and an increase in steroid dosage. Abscesses developed in the right buttock and left forearm.