A CLINICAL CASE OF TOURAINE-SOLENTE-GOLÉ SYNDROME
Abstract:
One of rare syndromes that can be found for one time only in one’s career is this case of
Pachydermoperiostosis (PDP) Touraine-Solente-Golé (TSG) syndrome. PDP or primary hypertrophic
osteoarthropathy (PHO) is a rare genetic disorder that affects both the bones and the skin. The disease
is also known under other terms: idiopathic hypertrophic osteoarthropathy or Touraine-Solente-Golé
syndrome. It is characterized by pachydermia (skin thickening), periostosis (excessive bone formation)
and tissue swelling with loss of normal angle between the nail and nail bed. This disease affects
relatively more men than women. It is an inherited disorder, autosomal dominant. It begins insidiously
at puberty. It is characterized by thickened skin of the face and extremities, thick and wavy scalp,
primary hypertrophic osteoarthropathy. Bone and inferior soft tissue proliferation resembles to that of
an elephant, hence the name of pachydermoperiostosis. After onset, the disease stabilizes after about 5
to 20 years. Lives of PDP patients can be severely damaged. Currently, the symptomatic treatment is
represented by NSAIDs and steroids, or surgical procedures. In 1868, PDP was first described by
Friedreich: “excessive growth of bones of the entire skeleton”. Touraine, Solente and Gole described
PDP as a primary form of bone disease - hypertrophic osteoarthropathy in 1935. Three forms can be
distinguished.
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