Musculoskeletal ImagingPachydermoperiostosis
primary hypertrophic
osteoarthropathy, inherited as an autosomal dominant trait and characterized by
clubbing of the digits in both the hands and the feet, enlargement of the
extremities owing to periarticular and osseous
proliferation, and painful and swollen joints. Complete, incomplete and
forme fruste varieties of this disorder occur. Patients with the complete syndrome have pachydermia, periostitis and cutis verticis gyrata; those with the the incomplete form exhibit sparing of the scalp; and the
forme fruste is manifested by pachydermia and minimal or absent periostitis. Frequently the disease progresses for about 10 years and is then arrested spontaneously. In some cases chronic disability may develop, however.
On radiographs, periostitis predominates. Irregular periosteal deposits appear about the metaphyses and epiphyses of the tubular bones, and bone thickening is most prominent in the tubular bones of the extremities (Fig.1). Poorly defined bone outgrowths in the epiphyses of tubular bones and sometimes also in the axial skeleton are especially characteristic of this disease. Diaphyseal expansion in tubular bones and sclerosis of the spongiosa in both appendicular and axial skeletal sites are evident. Other features are prominent sinuses, moderate enlargement of the mandible, and calcification and ossification of ligaments.
A syndrome resembling acromegaly has also been associated with pachydermoperiostosis, although the sella turcica is not enlarged and severe widening of the joint space does not occur.
DR/RB
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Frontal radiographs of the hand (a) and forearm (b) demonstrate enlargement of the tubular bones and prominence of the soft tissues.
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Pachydermoperiostosis, Fig.1 (a) | | Pachydermoperiostosis, Fig.1 (b) | |