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Publication
Caso dermatológico
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| 2.31 MB | Adobe PDF |
Advisor(s)
Abstract(s)
A esclerose tuberosa é uma síndrome neurocutânea, hereditária, autossómica dominante, caracterizada por manifestações pleomórficas envolvendo múltiplos órgãos, incluindo a pele. O diagnóstico de esclerose tuberosa é clínico. As lesões cutâneas mais comuns nestes doentes são: máculas hipopigmentadas, habitualmente com forma elíptica, os angiofibromas, tipicamente envolvendo a região malar e placas de “shagreen”, mais frequentemente localizadas à região inferior do tronco.
No caso descrito, a doente foi referenciada à consulta de dermatologia por uma lesão atípica de localização incomum, que demonstra a importância da observação de toda a pele nesta patologia.
Tuberous sclerosis is an inherited neurocutaneous disorder characterized by pleomorphic features involving many organ systems, including the skin. The diagnosis of tuberous sclerosis is clinical. The most common cutaneous lesions are hypopigmented macules, also known as ash-leaf spots, which are usually elliptic in shape, angiofibromas, which typically involve the malar regions of the face, and shagreen patches, most commonly present over the lower trunk. In the present case, the patient was referred to the dermatology department due to an atypical lesion with an uncommon location, revealing the importance of a whole body examination in patients with this disease.
Tuberous sclerosis is an inherited neurocutaneous disorder characterized by pleomorphic features involving many organ systems, including the skin. The diagnosis of tuberous sclerosis is clinical. The most common cutaneous lesions are hypopigmented macules, also known as ash-leaf spots, which are usually elliptic in shape, angiofibromas, which typically involve the malar regions of the face, and shagreen patches, most commonly present over the lower trunk. In the present case, the patient was referred to the dermatology department due to an atypical lesion with an uncommon location, revealing the importance of a whole body examination in patients with this disease.
Description
Keywords
Tuberous sclerosis Shagreen patch hypopigmented macules angiofibromas
Citation
Nascer e Crescer – Birth and Growth Medical Journal 2017; 26(2): 142-4
Publisher
Centro Hospitalar do Porto