Idiopathic Nonfamilial Aero-osteolysis with Cortical Defects and Mandibular Ramus Osteolysis

Abstract

A case is presented of progressive acro-osteolysis of the hands combined with progressive mandibular ramus osteolysis and multiple cortical defects. The features of acro-osteolysis are reviewed and classified.

Idiopathic non-familial Acro-osteolysis: A rare case report

“…[10] This is an extremely uncommon disease, and there are only very few published cases in the English literature. [1011] This variant may also be associated with Raynaud's phenomena.…”

“…[10] This is an extremely uncommon disease, and there are only very few published cases in the English literature. [1011] This variant may also be associated with Raynaud's phenomena. Only one case has been described to the best of our knowledge, with cutaneous lesions consisting of numerous yellow papules 2–4 mm in diameter and showing a linear distribution and coalescence into plaques, mainly on the arms.…”

Resorption of the mandibular condyles and coronoid processes in progressive systemic sclerosis (scleroderma)

“…Zarb and Carlsson (7) have also reported extensive destruction of the mandibular con-dyles in several patients with "scleroderma," although little clinical information is provided other than roentgenograms. Interestingly, both pairs of authors refer to previously reported cases of resorption of the mandibular condyles associated with idiopathic and polyvinylchloride associated acroosteolysis (14,15). Facial skin thickening occurred in at least 1 of these 2 instances of acroosteolysis (15).…”

Severe Hajdu-Cheney syndrome with upper airway obstruction

“…Reports of at least 40 other patients with this syndrome since that time were found in the radiological, pediatric, endocrine, oral surgery, and genetic literature [Greenberg and Street, 1957;Papavasiliou et al, 1960;Chawla, 1964;Dorst and McKusick, 1969;Herrmann et al, 1973;Matisonn and Ziady, 1973;Zugibe et al, 1974;Silverman et al, 1974;Weleber and Beals, 1976;Brown et al, 1976;Giula et al, 1976;Rosenmann et al, 1977;Williams, 1977;Elias et al, 1978;Vanek, 1978;Wendel andKemperdick, 1979 Iwaya et al, 1979;Kawamura et al, 1981;Zahran et al, 1984;Chodoroff et al, 1984;Blery et al, 1984;Niijima et al, 1984;Van Den Houten et al, 1985;Udell et al, 1986;Jacobson and Edekien, 1986;Kaler et al, 1990;Nunziata et al, 1990;Herscovici et al, 1990;Diren et al, 1990;Pellegrini and Widdowson, 1991;Kawamura et al, 1991;Adès et al, 1993;Zeman et al, 1994;O'Reilly and Shaw, 1994;Muller et al, 1994;Kaplan et al, 1995;Nishimura et al, 1996]. It became apparent that other signs were commonly found in HCS including a characteristic face described as broad with apparently lowset ears, hypertelorism, bushy eyebrows, lo...…”