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Case Report/Case Series |

Oral Pigmentation in McCune-Albright Syndrome

Dominique C. Pichard, MD1; Alison M. Boyce, MD2,3; Michael T. Collins, MD4; Edward W. Cowen, MD, MHSc5
[+] Author Affiliations
1Department of Dermatology, Georgetown University Hospital/Washington Hospital Center, Washington, DC
2Division of Endocrinology and Diabetes, Children’s National Medical Center, Washington, DC
3Bone Health Program, Division of Orthopaedics and Sports Medicine, Children’s National Medical Center, Washington, DC
4Skeletal Clinical Studies Unit, Craniofacial and Skeletal Diseases Branch, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, Maryland
5Center for Cancer Research, Dermatology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland
JAMA Dermatol. 2014;150(7):760-763. doi:10.1001/jamadermatol.2014.184.
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Importance  The differential diagnosis for oral lentigines includes several syndromes with important associated systemic findings. McCune-Albright syndrome (MAS), a mosaic condition associated with café au lait pigmentation, is not typically considered a mucosal lentiginosis syndrome. The clinical phenotype of MAS is variable because of mosaicism, but oral pigmentation developing in mid-childhood to early adulthood should be recognized as a clinical feature of MAS.

Observations  We present 4 patients with MAS who developed oral mucosal pigmentation during childhood or early adulthood. All patients had other characteristic findings of MAS including hyperfunctioning endocrinopathies, polyostotic fibrous dysplasia, and café au lait pigmentation.

Conclusions and Relevance  Oral pigmentation is an underrecognized finding in MAS and presents later in development compared with the other mucosal lentiginosis syndromes. The diagnosis of MAS is most commonly a clinical diagnosis because mutational analysis is challenging in mosaic conditions. Expanding the cutaneous phenotype to include oral pigmentation further characterizes the clinical findings in this mosaic condition, broadens the differential diagnosis of syndromes with oral pigmentation, and in some cases may aid in earlier diagnosis of MAS.

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Figure 1.
Case 1

A, Café au lait patch on the right lateral chest (age, 11 years). B, Oral mucosal pigmentation (age, 11 years). C, Oral mucosal pigmentation (age, 14 years).

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Figure 2.
Lip Pigmentation in Other Cases of McCune-Albright Syndrome

A, Case 2. Vermilion and mucosal pigmentation (age, 26 years). B, Case 3. Oral mucosal pigmentation (age, 7 years). C, Case 4. Lower vermilion lip pigmentation (age, 28 years).

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