Complete primary pachydermoperiostosis: A case report from Jordan and review of literature

Pachydermoperiostosis (PDP), also known as primary hypertrophic osteoarthropathy or Touraine‐Solente‐Gole syndrome, is a rare genetic disease with primary clinical features of pachydermia (thickening of skin) and periostosis (new bone formation). In 1935, three French dermatologists, Touraine et al, classified this condition as a familial disorder with three forms: complete (periostosis and pachyderma), incomplete (without pachyderma), and the forme fruste (pachydermia with minimal skeletal changes).1 The development of the disease usually starts during adolescence with gradual thickening of facial skin, which eventually resembles that of an elephant (pachyderm) and hence the name. The diagnosis should only be made when at least two out of a family history, hypertrophic skin changes, bone pain/radiographic changes, or clubbing are existing.2 The exact incidence is not known but estimated prevalence of the disease is 0.16%.3 We report a case of complete primary PDP that presented to Jordan University Hospital which is the second reported case of its kind in Jordan.4