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Lymphomatoid granulomatosis (LG) is a rare T cell rich, B cell non-Hodgkin's lymphoma which is difficult to diagnose. We present a patient with LG who demonstrated many of the difficulties in diagnosis and highlighted the importance of reviewing the diagnosis if treatment does not have the anticipated effect.A 49 year old male smoker presented to another hospital with a 3 month history of weight loss and sweats. A chest radiograph showed a mass in the left lower lobe and bronchoscopic examination revealed inflammation involving the left lower lobe bronchus. Washings were negative for organisms and malignancy. A computed tomographic (CT) scan showed a cavitating mass in the left lower lobe with multiple smaller opacities throughout both lung fields. Percutaneous fine needle aspiration of the mass showed inflammation with necrotic debris. He was discharged but presented 1 week later with fever and rigors and was transferred to this hospital.On arrival he had a cough productive of copious, foul smelling, purulent sputum. His temperature was 40˚C. There was no lymphadenopathy or hepatosplenomegaly. Neurological examination was normal. C-reactive protein (CRP) was 59 mg/l. Blood, urine and sputum cultures, white cell count, serum angiotensin converting enzyme, P-and C-ANCA, autoantibody screen, complement, test for HIV, viral screening, and brucella titres were all negative or normal. A repeat bronchoscopic examination was non-diagnostic. A repeat CT scan showed a 7 cm cavitating lesion in the left lower lobe with an air/fluid level and minor ''inflammatory changes'' in the right lung.Empirical treatment for a suspected lung abscess was commenced using intravenous benzylpenicillin, gentamicin, and metronidazole. Over the next 21 days his pyrexia settled, sputum cleared, and the CRP level normalised. The chest radiograph remained unchanged. Four weeks later his purulent sputum, fever, and raised CRP level recurred. Open lung biopsy was considered, but the thoracic surgical team declined because of the evidence for active infection. Antibacterial therapy was recommenced with intravenous cefotaxime and metronidazole. As before, there was a good clinical response but without radiological resolution. Further symptomatic relapse occurred once the drugs were withdrawn. Seven months after initial presentation he deteriorated further. A CT scan showed the left lower lobe cavity unchanged but there were now numerous nodular densities throughout the right lung (fig 1). Repeat bronchial biopsies showed abnormal lymphoid tissue characterised by a polymorphous lymphoid infiltrate containing scattered enlarged blast-like cells. These stained positively for CD20, a B cell marker, and immunoglobulin rearrangement studies showed clonality. These findings suggested lymphomatoid granulomatosis.He was treated with methylprednisolone, vinblastine, and cyclophosphamide. Within 2 weeks his dyspnoea, fever, and right sided radiological abnormalities resolved. The left lower lobe lesion reduced by 40% after three cycles of chemotherapy. Howev...

Two patients with pachydermoperiostosis were studied in whom the predominant features at presentation were severe and disabling knee and ankle joint pain in association with distal long bone pain. Analysis of synovial fluid from the knee joints showed non-inflammatory changes. In one patient a bicortical iliac crest bone biopsy specimen, taken after labelling with A radiological examination showed a periosteal reaction along the distal ulnae, tibiae, and fibulae and a bone scan using labelling with strontium 87 confirmed increased isotope uptake at these sites. A synovial biopsy sample from the right knee showed venous dilatation without inflammation whereas a biopsy sample of skin from the ankle showed gross dermal thickening with hypertrophy of the sweat glands. A biopsy sample ofthe tibial metaphysis, after labelling with demeclocycline, showed normal osteoid seams with minimal separation of the labelled lines, indicating that the hyperostosis was relatively inactive.Pachydermoperiostosis was diagnosed, the increased hydroxyproline excretion reflecting the increased metabolic rates of collagen and bone. In view of the severity of the patient's joint pain, which had responded poorly to treatment with NSAIDs, a trial with high doses of corticosteroids was thought to be justified (enteric coated prednisolone, 50 mg/day) and the effects were monitored biochemically using the excretion levels of urinary hydroxyproline. These decreased towards normal values rapidly but the symptoms continued intermittently and improved only gradually. By 1979 the patient still had disabling joint pain and his 24 hour urinary hydroxyproline excretion was still raised, at 32 mg/M2, but he was now working full time and the prednisolone, which had been gradually reduced, was discontinued. The patient subsequently continued to have joint symptoms, which were not progressive, and he remains otherwise well.PATIENT 2In 1989 a 17 year old healthy white boy developed severe pain and swelling of his knees and ankles after playing football. There was no