Etiology:

• Mutations of Gs-α gene (guanine nucleotide binder)
• Controls cAMP function in endocrine glands

Epidemiology:

• Monostotic – affects 1 bone
• Polyostotic – many bones
• Polyostotic syndromic

Clinical:

Triad:

1. Polyostotic fibrous dysplasia
2. Café au lait spots on skin and mucosa
3. Endocrine disturbances – precocious puberty

Diagnosis:

• X-ray: Fibrous dysplasia
• Lab: Hormone levels increase in blood test

Management:

• Drugs to inhibit bone resorption
• Surgery
• Hormone therapy
• Exercise – strengthen muscles surrounding fibrous dysplasia lesions
• Analgesics