Which syndrome is associated with cafe au lait macules?

McCune Albright syndrome is indeed associated with café au lait macules, which are flat, pigmented birthmarks that resemble the color of coffee with milk. This syndrome is a genetic disorder resulting from a mutation in the GNAS gene, leading to a triad of symptoms: fibrous dysplasia of bone, endocrine abnormalities (such as early puberty), and café au lait spots. The presence of these skin lesions can be an important clinical marker for diagnosing McCune Albright syndrome, as they often appear in a specific pattern, particularly in childhood.

In contrast, the other syndromes listed do not typically feature café au lait macules as a primary characteristic. Nevoid basal cell carcinoma syndrome generally presents with other skin lesions, such as basal cell carcinomas and jaw cysts. Turner syndrome features a variety of traits including short stature and gonadal dysgenesis, but not café au lait spots. Familial adenomatous polyposis is primarily characterized by the development of numerous polyps in the colon and rectum, with skin manifestations being quite different.

In summary, the association of café au lait macules with McCune Albright syndrome is key for recognizing the condition and understanding its implications for the patient's health.