Clinical, microscopic and imaging findings associated to McCune-Albright syndrome: report of two cases

McCune-Albright syndrome is characterized by the triad café-au-lait cutaneous spots, polyostotic fibrous dysplasia and endocrinopathies. This article presents two cases of McCune-Albright syndrome in a middle-aged woman and a young girl. Both patients presented café-au-lait spots on the face and oth...

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Published inBrazilian dental journal Vol. 19; no. 2; p. 165
Main Authors Xavier, Samuel Porfírio, Ribeiro, Michel Campos, Sicchieri, Luciana Gonçalves, Brentegani, Luiz Guilherme, Lacerda, Suzie Aparecida
Format Journal Article
LanguageEnglish
Published Brazil 2008
Subjects
Bone Density - physiology
Cell Nucleus - pathology
Child
Collagen
Diagnosis, Differential
Facial Asymmetry - diagnosis
Facial Bones - pathology
Female
Fibroblasts - pathology
Fibrous Dysplasia, Polyostotic - diagnosis
Fibrous Dysplasia, Polyostotic - pathology
Follow-Up Studies
Humans
Mandibular Diseases - diagnosis
Mandibular Diseases - pathology
Middle Aged
Radiography, Panoramic
Skull - pathology
Tomography, X-Ray Computed
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Summary:McCune-Albright syndrome is characterized by the triad café-au-lait cutaneous spots, polyostotic fibrous dysplasia and endocrinopathies. This article presents two cases of McCune-Albright syndrome in a middle-aged woman and a young girl. Both patients presented café-au-lait spots on the face and other parts of the body and expansion of the mandible with radiopaque-radiolucent areas with ground-glass radiographic appearance, and were diagnosed as having fibrous dysplasia and endocrine disorders. The patient of Case 1 had fibrous dysplasia on the upper and lower limbs, thorax, face and cranium, early puberty, hyperglycemia, hyperthyroidism and high serum alkaline phosphatase levels. The patient of Case 2 presented lesions on the upper limbs and evident endocrine disorders. In both cases presented in this article, the initial exam was made because of the mandibular lesion. However, a diagnosis of fibrous dysplasia must lead to investigation of the involvement of other bones, characterizing polyostotic fibrous dysplasia, which is manifested in a number of diseases. An accurate differential diagnosis is mandatory to determine the best treatment approach for each case.
ISSN:1806-4760