= OSTEODERMOPATHIA HYPERTROPHICANS (TOURAINE-SOLENTE-GOLE) = PRIMARY HYPERTROPHIC OSTEOARTHROPATHY

Autosomal dominant

Age: 3–38 years with progression into late 20s / 30s; M >>F

• large skin folds of face + scalp

Location: epiphyses + diametaphyseal region of tubular bones; distal third of bones of legs + forearms (early); distal phalanges rarely involved

• enlargement of paranasal sinuses
• irregular periosteal proliferation of phalanges + distal long bones (hand + feet) beginning in epiphyseal region at tendon / ligament insertions
• thick cortex, BUT NO narrowing of medulla
• clubbing
• may have acroosteolysis

Prognosis: self-limiting = progression ceases after several years

DDx: hypertrophic pulmonary osteoarthropathy, thyroid acropachy