The Albright syndrome

The Albright syndrome (F. Albright) - a disease characterized by the triad of symptoms: fibrous osteodysplastic, lesions of cutaneous hyperpigmentation and premature puberty. Much more common in women.
The etiology of Albright syndrome is unknown; assume neurogenic effects and endocrine changes caused by congenital changes in the hypothalamic region, with premature products gonadotroponah hormones of the anterior pituitary gland because of sclerosis and giperostos skull base" mainly in the field of the Turkish saddle (Fig).
The growth and maturity of the skeleton (appearance and fusion of ossification) can significantly ahead of normal levels. As a result, the media Albright syndrome in childhood may be higher, and after the premature fusion of the epiphyses - below normal. To advance the development of the skeletal accompanies premature puberty is sometimes blurred, rarely significant, when the menstrual function can occur in the early years, and sporadic menorrhagia - even in the first months of life. Premature puberty in boys rare.
The hyperpigmentation of the skin with O. S. observed on the torso, thighs, less often on the neck, the shins. They look like large fields or individual and merging spots with diameter from 2 to 20 cm or more, irregular shape with cut edges or submitted by clusters of freckles from pale-yellow to brown color. Sometimes these sites are already visible at birth, but at the small size and the color can be viewed. Localization skin changes do not always coincide with bone lesions. Hyperpigmented skin are not elevated.
When Albright syndrome, and there are others reject - ray, diabetes, vices vessels, cartilaginous osteodysplastic and other Biochemical indices in O. C. normal; sometimes increased alkaline phosphatase activity (up to 20% Bogunskogo). Among the complications of fibrous dysplasia, specific O. S., meet optic nerve atrophy and exophthalmos as a consequence of giperostos region of the Turkish saddle and orbit.
The prognosis for O. S. favorable, but possible pathological fractures and deformation of bones and in rare cases zlokacestvennoe fibrous tricks.
Differential diagnosis and treatment of bone component O. S. see Osteodysplastic. Skin and endocrine aspects in case of O. S. irreversible.

Seal the bone structure of the skull base, mainly in the field of the Turkish saddle.