A Case of Albright's Syndrome.
- Author:
Yong Woo CINN
- Publication Type:Case Report
- MeSH:
Acromegaly;
Alkaline Phosphatase;
Child;
Child, Preschool;
Congenital Abnormalities;
Cushing Syndrome;
Extremities;
Female;
Femur;
Fibrous Dysplasia, Polyostotic*;
Fibula;
Fractures, Spontaneous;
Gait;
Humans;
Humerus;
Hyperthyroidism;
Male;
Optic Nerve;
Pelvis;
Puberty, Precocious;
Scapula;
Skeleton;
Skin;
Skull Base;
Sphenoid Bone;
Spine;
Tibia;
Vision, Low
- From:Korean Journal of Dermatology
1984;22(1):111-115
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Albright's syndrome consists of large pigmented macules of the skin and fibrodysplasia of the bones. The full syndrome with precocious puberty occurs particularly in girls. Other endocrinopathies including Cushing syndrome, hyperthyroidism and acromegaly can be accompanied. A case of Albright's syndrome is reported. The patient was a 4-year-old male child, who was born with multiple, dark brownish, irregularly bordered macules on the trunk and extremities. Deformities of facial skeleton and limping gait were noticed at the age of 2 years. He had pathological fractures twice, at the age of 3 and 4 years, respectively, due to fibrodysplasia of both femur bones. Especially, involvement of the sphenoid bones caused compression of the optic nerves and diminished vision of both eyes. On radiologic examinations, polyostotic fibrous dysplasia were noticed on the humerus, scapula, femur, tibia, fibula, pelvis, vertebra and skull base. The level of alkaline phosphatase was highly elevated.