A family with a variant form of primary hypertrophic osteoarthropathy restricted to the lower extremities

Abstract

We report a case of a family with a variant form of primary hypertrophic osteoarthropathy (HOA) restricted to the lower extremities without digital clubbing or cutaneous changes. Three family members suffered from pain, swelling and hyperhidrosis of both feet. X-rays showed destruction and osteoproliferative changes of the metatarsal bones with periostal hyperostosis close to the talus. There was an increased 99m-Tc-MDP uptake in the early phase of bone scintigraphy. Fibrosis of the marrow with stimulated osteoclastic resorption and the presence of detritus synovialitis were visible in a bone and joint biopsy. All known infectious, neurologic, metabolic and malignant diseases, which affect the bone and joints, were excluded.