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Abstract #22023 Published in IGR 10-4
Phakomatosis pigmentovascularis type IIA: About a case at CHU of treichville associated to a Sturge-Weber syndroma and to a type 6 neurofibromatosis
Ecra E; Enoh S; Kouame AK; Gbery I; Sangare A; Kaloga M; Azagoh K; Boni S; Yoboue P; Kassi KNouvelles Dermatologiques 2008; 27: 333-335
Phakomatosis pigmentovascularis is a rare congenital dermatosis. It is a neuro-oculo-cutaneous dysembryoplasia. The authors report the case of a 4-month-old new-born baby with epilepsia. Clinical signs are a Sturge-Weber angiomatosis with a systematized port-wine stain made by an erythema of the right hemiface going broadly to the left, linked to a homolateral involvement of the thorax and the upper limbs, a craniofacial dysmorphy and a glaucoma. Electroencephalogram shows a stage II and III bilateral activity of sleep with numerous physiological spindles of sleep. Head scanner shows a diffuse cortico-sous-cortical atrophia and a cerebellar atrophia. The associated melanosis is characterized by an oculo-cutaneous involvement with dark iris and a giant nevus made of pigmentation in roughly symmetrical tablecloth of both lower limb and buttocks. Finally, café-au-lait spots are associated on dorsum. The authors underline therefore the necessity for clinical, electrical and radiological investigations to every new-born baby bearing facial port-wine stain with neurological signs for a better taking care. LA: French
Dr. E. Ecra, Service de Dermatologie, CHU de Treichville, 15 BP 4, Abidjan 15, Cote d'Ivoire