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Pachydermoperiostosis (Idiopathic Clubbing and Periostosis)

1965; Massachusetts Medical Society; Volume: 272; Issue: 18 Linguagem: Inglês

10.1056/nejm196505062721801

1533-4406

David L. Rimoin,

Connective tissue disorders research

PACHYDERMOPERIOSTOSIS (idiopathic hypertrophic osteoarthropathy) is a syndrome characterized by clubbing of the digits, periosteal new bone formation, especially over the distal ends of the long bones, and coarsening of the facial features, with thickening, furrowing and oiliness of the skin of the face and forehead.1 The furrowing may also involve the scalp, where it is known as cutis verticis gyrata.The skin exhibits marked seborrheic hyperplasia with wide-open sebaceous pores filled with plugs of sebum. Ptosis, caused by hypertrophy of the eyelids, may be so severe as to obstruct vision.2 , 3 The skin over the hands and feet is often thickened . . .