A boy with enlarging hands and feet

A teenager presented with a complaint of progressive headache and painful swelling of the wrists. He had spade-like hands and feet along with increased heel pad thickness, increased shoe and ring tightening, characteristic coarse facial features, and a large fleshy nose that had developed over the past few months

He was initially screened with a computed tomography (CT) scan of the head and serum IGF-1, both of which turned out to be normal. Finally, an x ray of the wrist revealed the diagnosis (fig 1).

Figure 1.

Periostitis: symmetric osseous thickening.

Pachydermoperiostosis is also known as primary hypertrophic osteoarthropathy. The clinical manifestations of patients with pachydermoperiostosis vary. These manifestations include those of the complete form (pachydermia, periostitis, cutis vertices gyrata) and those of the incomplete form (sparing of the scalp, forme fruste, pachydermia with minimal or absent periostitis). Enlargement of the hands and feet, clubbing of the distal fingers and toes, coarsening of the skin of the face and scalp with ptosis, furrowing and oiliness of cutaneous tissue, excessive sweating, fatigability, pain in bones and joints, hepatosplenomegaly anaemia, and endocrine abnormalities may be seen.^1,2

The predominant radiographic feature of pachydermoperiostitis is periostitis, which is depicted as symmetric osseous thickening. Periostitis mostly affects the tubular bones of the limbs, especially the radius, ulna, tibia, and fibula, although the pelvis, carpus, tarsus, metacarpals, metatarsals, and phalanges can be involved.

Periosteal proliferation is usually dishevelled and associated with irregular excrescences and diaphyseal expansion. Periosteal proliferation begins in the epiphyseal region at the tendon–muscle attachment. Rarely, thickening of the calvarium and skull base is seen.³