Alopecia with fibrous dysplasia and osteomas of skin. A sign of polyostotic fibrous dysplasia.
Localized alopecia of the scalp proved to be the cutaneous sign of underlying polyostotic fibrous dysplasia (Albright disease). Histologically, the hair follicles had been replaced by a strange coil of fibrous tissue. Some of these follicular sites showed ossification so prominent as to be evident in soft tissue reoentgenograms. In this patient, the skin itself showed a fibrous dysplasia analogous to that which has always been identified as occurring within the bones in Albright disease. In her skin, it is the hair follicle that is the site of this dysplasia. The appearance of ossification in the skin and fibrous dysplasia in the bone would seem to demonstrate a delicate equilibrial balance between fibrous and osteal tissue in organs of a common mesenchymal origin. Localized connective tissue stromal dysplasia is believed to play a role in the pathogenesis of not only the bony, but aslo the pigmentary and endocrine abnormalities of the disease.
D000505:Alopecia / Q000209:etiology* / Q000473:pathology; D005260:Female; D005350:Fibroma / Q000209:etiology* / Q000473:pathology; D005357:Fibrous Dysplasia of Bone / Q000175:diagnosis*; D005358:Fibrous Dysplasia, Monostotic / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D006801:Humans; D008875:Middle Aged; D012878:Skin Neoplasms / Q000209:etiology* / Q000473:pathology