pachydermoperiostosis


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pachydermoperiostosis

 [pak″e-der″mo-per″e-os-to´sis]
a condition believed to be inherited as an autosomal dominant trait, chiefly characterized by thickening of the skin of the head and distal extremities, deep folds and furrows of the skin of the forehead, cheeks, and scalp, seborrhea, hyperhidrosis, periostosis of the long bones, digital clubbing, and spadelike enlargement of the hands and feet.

pach·y·der·mo·per·i·os·to·sis

(pak'ē-der'mō-per'ē-os-tō'sis), [MIM*167100]
A syndrome of clubbing of the digits, periosteal new bone formation, especially over the distal ends of the long bones (idiopathic hypertrophic osteoarthropathy), and coarsening of the facial features with thickening, furrowing, and oiliness of the skin of the face and forehead (cutis verticis gyrata); there is seborrheic hyperplasia with open sebaceous pores filled with plugs of sebum; often of autosomal dominant inheritance, usually more severe in males.
Synonym(s): acropachyderma
[pachy- + G. derma, skin, + periostosis]

pachydermoperiostosis

/pachy·der·mo·peri·os·to·sis/ (-der″mo-per″e-os-to´sis) pachyderma affecting the face and scalp, thickening of the bones of the distal limbs, and acropachy.

pachydermoperiostosis

[-dur′moper′ē·ostō′sis]
a syndrome characterized by a thickening and folding of the facial skin, clubbing of the fingers, and new bone formation over the ends of the long bones.

pachydermoperiostosis

An AD condition characterized by induration of skin in natural folds, accentuation of creases of face and scalp, finger clubbing and periostosis of long bones; acquired pachydermoperiostosis is linked to bronchogenic carcinoma, termed hypertrophic–pulmonary osteoarthropathy, see there.
References in periodicals archive ?
The action of prostaglandins on ciliary hypertrichosis: A case report of pachydermoperiostosis.
Pachydermoperiostosis - a case report of complete form and literature review.
Current treatment modalities for pachydermoperiostosis are limited.
in the differential diagnosis of acromegaly, the possibility of pachydermoperiostosis has to be kept in mind, because treatment modalities differ between these two diseases.
Pachydermoperiostosis might be confused with many diseases, including acromegaly.
Blepharoptosis is often not severe in pachydermoperiostosis.
Pachydermoperiostosis (idiopathic hypertrophic osteoarthropathy).
Pachydermoperiostosis (syndrome of Touraine, Solente and Gole).
DISCUSSION: Pachydermoperiostosis or primary hypertrophic osteoarthropathy is a rare hereditary disorder, which was first described by Friedreich in 1868.
2) Pachydermoperiostosis is a familial disorder, inherited autosomal dominant pattern.
Pachydermoperiostosis is very rare, and its clinical and radiological presentations can be confused with those of syphilitic periostitis, psoriatic onychopachydermoperiostitis (POPP), Paget's disease, and, especially, acromegaly.
Pachydermoperiostosis cases mimicking acromegaly have been reported so as to bring them to the attention of practitioners.