Hereditary hemorrhagic teleangiectasia (HHT) is an inherited rare disorder in which patients may have dermatological and systemic involvement. The nondermatological involvement of HHT regards eyes, gastrointestinal apparatus, liver, lung and CNS. Telangiectasia represents the typical and always present skin lesion of HHT. Theoretically the dermatological diagnosis of the HHT is easy. However, given the large number of diseases characterized by telangiectasia, there are some problems of differential diagnosis. In this paper we present 5 new cases of HHT together with a review of literature. The authors selected five dermatological signs useful for the diagnosis of HHT syndrome and for discrimination among the large number of diseases characterized by telangiectasia. Following these dermatological criteria the clinical diagnosis of HHT is easier and the patient may be submitted to first and second level examination in order to discover a systemic lifethreatening involvement.
|Translated title of the contribution||The dermatological contribution to the diagnosis of hereditary hemorrhagic telangiectasia syndrome|
|Number of pages||7|
|Journal||Giornale Italiano di Dermatologia e Venereologia|
|Publication status||Published - 1996|
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