[Pachydermoperiostosis--report of a case and review of 121 Japanese cases].

Abstract

A case of 23 years old man with idiopathic pachydermoperiostosis is reported. He showed cutis verticis gyrata, clubbed fingers and periosteal new bone formation without any causative basic disorder. His serum level of FSH, LH, estradiol and estriol were elevated, but their significance was not clear. Histological examination of the skin from the forehead revealed sebaceous hyperplasia and dermal thickening, where deposit of alcian blue and colloidal iron positive substance were detected. The deformed forehead and eyelids were corrected by plastic surgery. One hundred and twenty one cases of pachydermoperiostosis reported so far in Japan are briefly reviewed. Most of them were male (94.1%), about one fourth had a family history. The principal features are: clubbing of the digits (88.4%), periosteal new bone formation (94.1%), coarsening of the facial features with furrowing of the skin of the face (72.7%) and cutis verticalis gyrata (59.5%). Arthralgia (40.5%), hyperhidrosis of the feet and hands (44.6%), gastric hypertrophy (8 cases), gastric ulcers (5 cases) and endocrine abnormalities (17 cases) were also reported.