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 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.
Xavier
Samuel Porfírio
SP
Department of Oral and Maxillofacial Surgery and Traumatology and Periodontics, Dental School of Ribeirão Preto, University of São Paulo, Ribeirão Preto, SP, Brazil.
Ribeiro
Michel Campos
MC
Sicchieri
Luciana Gonçalves
LG
Brentegani
Luiz Guilherme
LG
Lacerda
Suzie Aparecida
SA
D015519:Bone Density / Q000502:physiology; D002467:Cell Nucleus / Q000473:pathology; D002648:Child; D003094:Collagen; D003937:Diagnosis, Differential; D005146:Facial Asymmetry / Q000175:diagnosis; D005147:Facial Bones / Q000473:pathology; D005260:Female; D005347:Fibroblasts / Q000473:pathology; D005359:Fibrous Dysplasia, Polyostotic / Q000175:diagnosis* / Q000473:pathology; D005500:Follow-Up Studies; D006801:Humans; D008336:Mandibular Diseases / Q000175:diagnosis* / Q000473:pathology; D008875:Middle Aged; D011862:Radiography, Panoramic; D012886:Skull / Q000473:pathology; D014057:Tomography, X-Ray Computed
D003094:Collagen