Bone abnormalities and severe arthritis in pachydermoperiostosis.

Abstract

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

Hypertrophic osteopathy in a dog associated with intra-thoracic lesions: a case report and a review

“…These bone changes are not a primary neoplasia or metastatic bone lesions (Lenehan and Fetter, 1985;Johnson et al, 1995;Morrison, 2002;Montgomery, 2003;Piermattei et al, 2006). Although the joints are not affected, the range of joint motion may become diminished because of periarticular soft tissue swelling (Montgomery, 2003;Piermattei et al, 2006); however, inflammatory changes in the synovial membrane of swollen painful joints were reported in humans (Cooper et al, 1992).…”

A case of pachydermoperiostosis treated by oral administration of a bisphosphonate and arthroscopic synovectomy

“…Risedronate sodium has been reported to work on osteoclasts, resulting in a decrease in urinary NTx. 10 Cooper et al 11 reported an increased number of osteoclasts in the bone of PDP patients. It is considered that risedronate sodium inhibited the osteoclasts in the bones of our PDP patient.…”

A case of pachydermoperiostosis associated with arthritis

“…In almost 40% of patients, this disease is complicated by joint effusion, but the symptoms are not particularly severe 8 and only a few cases experience severe joint pain. 9 Histological examinations of synovia in those patients showed hypercellularity and vascular thickening or venous dilatation without inflammation. 9,10 In addition, no inflama b c d matory signs were detected in the synovial fluid.…”

“…9 Histological examinations of synovia in those patients showed hypercellularity and vascular thickening or venous dilatation without inflammation. 9,10 In addition, no inflama b c d matory signs were detected in the synovial fluid. In some cases, patient's presented with acute-onset arthralgia derived from pseudogout.…”