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- Pierre-Marie-Bamberger syndrome
- hypertrophic osteoarthropathy
- paraneoplastic vasculitis
- lung carcinoma
Paraneoplastic syndromes may occur as the first clinical symptom at an early stage of malignant diseases in about 7–10% of cases.1 As presented here, intensive investigations may be necessary to uncover an early curable tumour.
A 72 year old man presented with multiple purpuric and necrotising skin eruptions (sized 2–20 mm) covering mainly the extremities and, to a lesser extent, the trunk. He had noticed a weight loss of 12 kg in the past 2 months. There was a history of smoking with chronic obstructive pulmonary disease and partial colectomy because of a benign adenoma. Laboratory investigations showed a normal erythrocyte sedimentation rate (9 mm/1st h), but C reactive protein was raised (43 mg/l). Serological markers, such as antinuclear antibodies, antineutrophil cytoplasmic antibodies, cryoglobulins, and hepatitis B and hepatitis C were excluded. A diagnostic procedure was performed to exclude a neoplasm, but, chest radiography, abdominal ultrasonography, gastroscopy, and colonoscopy were normal. Prednisone was given at a dose of 1 mg/kg body weight, with gradual improvement of the cutaneous vasculitis.
During the residential period the patient complained of pain in the region of the distal femur and the ankle bilaterally. Musculoskeletal ultrasound of the knee joints showed an effusion of 6 ml from the right and 3 ml from the left, without signs of synovitis, but discrete osteophytes. However, at the surface of the distal femoral bone echogenic irregular foci bilaterally with distal acoustic shadowing were seen, suggesting periosteal calcifications (fig 1A). An x ray examination confirmed symmetric periosteal reactions of the femora of both sides (fig 1B), compatible with periostitis. Inflammatory activity was shown by a significant enhancement in both distal femora in a three phase bone scan with technetium-99m DPD (fig 1C).
Even though there was no sign of a tumour on the standard chest radiographs a computed tomographic scan of the lung was performed. A suspect lesion of 1.9 cm in diameter in the right lung was seen (fig 1D). A transthoracic puncture showed a highly differentiated adenocarcinoma of the lung. No metastasis was seen. The patient was referred to a thoracic surgeon and the tumour was excised. Re-evaluation 6 months after excision of the tumour showed complete remission of bone pain and vasculitis without immunosuppressive treatment.
Our patient presented with two paraneoplastic syndromes: hypertrophic osteoarthropathy and cutaneous vasculitis.
Hypertrophic osteoarthropathy is most commonly seen in Pierre-Marie-Bamberger’s syndrome. This syndrome is characterised by the concurrence of clubbed fingers, arthralgia, and painful periostitis of the extremities, and occurs in pulmonary, cardiac, hepatic, or intestinal diseases.2 Rarely, periostitis may be the only manifestation of Pierre-Marie-Bamberger’s syndrome3 as in our patient. The periostitis is the result of subperiosteal bone formation, occurring mainly on the distal diaphysis of the long bones. Effusions into the large joints with little inflammatory cell exudation are a frequent finding. The diagnosis of periostitis is usually made by radiography and by bone scan. As we showed, musculoskeletal ultrasound may also visualise periostitis, but it is needed especially to look for periosteal lesions far from the adjacent joints. Table 1 presents other conditions associated with periostitis.
Non-small lung cell cancer is the most common malignancy associated with both Pierre-Marie-Bamberger’s syndrome and cutaneous vasculitis.4,5 However, hypertrophic osteoarthropathy is more common (4.9% in one study) than paraneoplastic vasculitis in patients with lung cancer.6,7 Differential diagnosis includes polyarteritis nodosa and systemic lupus erythematosus; both have been associated with periosteal reactions and vasculitis in anecdotal reports.8,9,10 However, as demonstrated by our case, computed tomography of the lung should be performed in the presence of unexplained vasculitis or periostitis, or both, even if standard chest radiography of the lung shows no pathological findings.
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