Clinical, microscopic and imaging findings associated to Mccune-Albright syndrome: report of two cases
Braz. dent. j
;
19(2): 165-170, 2008. ilus
Article
in English
| LILACS
| ID: lil-484955
ABSTRACT
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.
RESUMO
A Síndrome de McCune-Albright é caracterizada pela tríade manchas café-com-leite na pele, displasia fibrosa poliostótica e endocrinopatias. Este artigo apresenta dois casos de Síndrome de McCune-Albright em uma mulher de meia idade e em uma menina. Ambos apresentavam manchas café-com-leite na face e em outras partes do corpo, expansão da mandíbula com área radiopaca-radiolúcida com aspecto de vidro despolido na radiografia, com o diagnóstico de displasia fibrosa, e desordens endócrinas. O paciente do Caso 1 apresentava displasia fibrosa nos membros inferiores e superiores, tórax, face e crânio, puberdade precoce, hiperglicemia, hipertireoidismo e elevação da fosfatase alcalina no sangue. O paciente do Caso 2 apresentava lesões nos membros superiores e desordem endócrina evidente. Em ambos os casos apresentados neste artigo, o exame inicial foi realizado devido à lesão mandibular. Contudo, o diagnóstico de displasia fibrosa deve conduzir a pesquisa de envolvimento de outros ossos, caracterizando a displasia fibrosa poliostótica, que se manifesta em um grande número de doenças. O diagnóstico diferencial preciso é fundamental para determinar o tratamento adequado a cada caso.
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Mandibular Diseases
/
Fibrous Dysplasia, Polyostotic
Type of study:
Diagnostic study
/
Observational study
/
Prognostic study
/
Risk factors
Limits:
Child
/
Female
/
Humans
Language:
English
Journal:
Braz. dent. j
Journal subject:
Dentistry
Year:
2008
Type:
Article
Affiliation country:
Brazil
Institution/Affiliation country:
University of São Paulo/BR
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