[Pachydermoperiostosis. Clinical aspects, classification and pathogenesis].
There are 2 different forms of the rare disorder of connective tissue "pachydermoperiostosis": a primary form called Touraine-Solente-Gole (TSG) syndrome, and a secondary one, called Bamberger-Pierre-Marie syndrome (BPM). The manifestations of the two forms are very similar, but differential diagnosis is possible because BPM syndrome occurs in association with certain diseases of internal organs and effective treatment of these is followed by regression of the skin manifestations. Therefore, it seems reasonable merely to refer to primary and secondary forms of pachydermoperiostosis, rather than distinctly named syndromes. The pathogenesis of pachydermoperiostosis is unknown. Obviously a hereditary disturbance of metabolism in the connective tissue is involved, and manifestation of the disease can be either primary or secondary as a result of still unknown triggering factors.
Lindmaier
A
A
II. Universitäts-Hautklinik Wien.
Raff
M
M
Seidl
G
G
Jurecka
W
W
D000328:Adult; D001485:Basement Membrane / Q000473:pathology; D003094:Collagen / Q000378:metabolism; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D010004:Osteoarthropathy, Primary Hypertrophic / Q000175:diagnosis* / Q000473:pathology; D012867:Skin / Q000473:pathology; D013545:Sweat Glands / Q000473:pathology
D003094:Collagen
Pachydermoperiostose. Klinik, Klassifikation und Pathogenese.