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

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 ?
Rheumatologic manifestations of pachydermoperiostosis and preliminary experience with bisphosphonates.
The action of prostaglandins on ciliary hypertrichosis: A case report of pachydermoperiostosis. Int J Trichology.
recognized three forms of pachydermoperiostosis: a complete form characterized by both pachyderma (thickening of the skin) and periostitis, an incomplete form without pachyderma and a third form having pachyderma with minimal skin changes (Forme Fruste) [14].
Rhee, "Identification of the mutations in the prostaglandin transporter gene, SLCO2A1 and clinical characterization in Korean patients with pachydermoperiostosis," Journal of Korean Medical Science, vol.
In 1868, Friedrich described pachydermoperiostosis in two young brothers.
Current treatment modalities for pachydermoperiostosis are limited.
The most common ocular associations of pachydermoperiostosis are blepharoptosis and tarsitis.
Incomplete pachydermoperiostosis. Proc Roy Soc Med 1968; 61: 239-240.
DISCUSSION: Pachydermoperiostosis or primary hypertrophic osteoarthropathy is a rare hereditary disorder, which was first described by Friedreich in 1868.(1,2) Matucci-cerinic et al.
Pachydermoperiostosis is a rare hereditary disorder which mainly appears as an autosomal dominant pattern with variable penetration.
Hypertrophy of the feet and ankles presenting in primary hypertrophic osteoarthropathy or pachydermoperiostosis: a case report Journal of Medical Reports 2012 6: 31.
Cutis verticis gyrata as a clinical manifestation of Touraine-Solente-Gole' syndrome (pachydermoperiostosis).