oa South African Medical Journal - Hereditary haemorrhagic telangiectasia : report of a Bantu case



The first Bantu case of hereditary haemorrhagic telangiectasia is reported. All three features of the disease were present, namely, repeated haemorrhage from the nose, telangiectatic lesions and evidence that the disease had been inherited. The blood picture showed an anaemia, probably secondary to the haemorrhages and an eosinophilia which was ascribed to a concomitant active bilharzia. Splenomegaly was present, and although this may be found in hereditary haemorrhagic telangiectasia, the patient had come from an endemic malaria district. An exfoliative cheilitis was present, the significance of which was not clear. The histology of the lesions as descriptionbed in the literature is briefly reviewed. Histological study of a nodular telangiectatic lesion from the patient showed gross dilatation of papillary and sub-papillary capillaries. In the deep layer of the corium beneath the middle of the lesion, a dense vascular network was noted, consisting of venules and arterioles, the latter showing well-developed muscular coats. The interstitial collagen and elastic tissues were normal. No information regarding the pathogenesis of the condition could be obtained from study of this specimen. No support could be found for the hypotheses that the vascular dilatations were due either to connective tissue degeneration or to occlusion of venules.


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