This 15 year old girl was noted to have large café-au-lait macules (CALMs) on her left buttock, thigh, and leg at birth. Growth and development have been normal. She has normal menstrual periods, and ovarian ultrasound and bone radiographs were normal. The presence of large congenital CALMs should suggest the possibility of McCune-Albright syndrome, neurofibromatosis (NF), and other disorders associated with CALMs. McCune-Albright syndrome is a sporadic disease, characterized by polyostotic fibrous dysplasia, CALMs , and hyperfunctional endocrinopathies. Many of the reported patients lack one feature of the triad. Macules occur at birth, but new lesions may appear, becoming more distinct during the first few months of life as they enlarge. CALMs are often unilateral with jagged borders, and present over the underlying bone defects or on the same side of the body. As opposed to NF-1, lesions are fewer, larger, and darker, but it is often difficult to differentiate the two, based on the number, size, and shape of theCALMs. Although this girl may only have isolated CALMs, she will continue to be monitored for the development of signs and symptoms of McCune-albright syndrome and NF1. Reference: The diagnostic value of café-au-lait macules J Am Acad Dermatol 1999;40(pt 1):877-92.
Description
crisply demarcated uniform brown irregularly shaped patches
