They found that striking overgrowth of tissues occurred after surgical operations. (1990) provided follow-up on the severely affected child originally reported by Malamitsi-Puchner et al.
![proteus syndrome skeleton proteus syndrome skeleton](https://thegeminicompany.com/wp-content/uploads/2020/01/Elephant-Man-right-2.jpg)
(1987) reported 6 cases, 11 cases, and 1 case, respectively. (The hypothesis has been proved in the case of the latter condition.) Rescue of a lethal genotype by chimerism with normal embryos ( Bennett, 1978) is an experimental model of this mode of inheritance. He suggested this mechanism also for Schimmelpenning-Feuerstein-Mims syndrome ( 163200) and the McCune-Albright syndrome ( 174800). Happle (1986) pointed out that the lesions follow the lines of Blaschko and suggested that the cause is a dominant lethal gene surviving by mosaicism. (1985) noted that mean paternal age at the time of birth of 10 of the patients was 30 (range 23 to 40), which is probably not significantly elevated. Some similarities to the Bannayan-Zonana syndrome (see 153480) and linear sebaceous nevus syndrome ( 163200) were noted. Laparotomy at age 6 for acute abdominal pain showed right iliac fossa lipomatosis and twisted necrotic mesenteric fat as the presumed cause of pain. One, a 7-year-old boy, was noted at age 3 to have a conjunctival dermoid. (1985) reported 2 cases both had abdominal and pelvic lipomatosis.
#Proteus syndrome skeleton skin
Burgio and Wiedemann (1984) found that the skin changes are papillomatous epidermal nevi.Ĭosta et al. The disorder might be confused with the Klippel-Trenaunay-Weber syndrome ( 149000) and with Ollier disease ( 166000) and Maffucci syndrome ( 614569). (1983) suggested that the patient reported by Temtamy and Rogers (1976) and probably also the patient of Graetz (1928) may have had this disorder. They named the syndrome for the Greek god Proteus, 'the polymorphous,' who could change his shape at will to avoid capture. The authors considered the disorder to fall into the category of congenital hamartomatous disorders and to be 'undoubtedly genetically determined,' perhaps as an autosomal dominant disorder.
![proteus syndrome skeleton proteus syndrome skeleton](http://pubs.sciepub.com/ajmcr/2/7/2/image/fig6.png)
(1983) described a 'new' syndrome in 4 unrelated boys with the combination of partial gigantism of the hands and/or feet, nevi, hemihypertrophy, subcutaneous tumors, macrocephaly or other skull anomalies, and possible accelerated growth and visceral affections.