[Phakomatosis pigmentovascularis cesioflammea: a case report].

Autor:	Viada Peláez MC; Servicio de Dermatología, Hospital Nacional de Pediatría 'Prof. Dr. J. P. Garrahan', Ciudad Autónoma de Buenos Aires. candeviada@hotmail.com., Stefano PC; Servicio de Dermatología, Hospital Nacional de Pediatría 'Prof. Dr. J. P. Garrahan', Ciudad Autónoma de Buenos Aires., Cirio A; Servicio de Dermatología, Hospital Nacional de Pediatría 'Prof. Dr. J. P. Garrahan', Ciudad Autónoma de Buenos Aires., Cervini AB; Servicio de Dermatología, Hospital Nacional de Pediatría 'Prof. Dr. J. P. Garrahan', Ciudad Autónoma de Buenos Aires.
Jazyk:	Spanish; Castilian
Zdroj:	Archivos argentinos de pediatria [Arch Argent Pediatr] 2018 Feb 01; Vol. 116 (1), pp. e121-e124.
DOI:	10.5546/aap.2018.e121
Abstrakt:	Phakomatosis pigmentovascularis (PPV) is a syndrome characterized by the association of a vascular nevus with a congenital pigmented lesion (epidermal nevus, nevus spilus, and dermal melanocytosis). There are different types of PPV according to the pigmentary nevus associated with the vascular malformation. Patients may present only the cutaneous condition or have systemic manifestations, among them, trauma, neurological and ophthalmological disorders. We report the case of a 1-year-old girl who had congenital glaucoma. On examination, we identified facial paralysis, bilateral ocular melanosis, segmental capillary vascular malformation on the face as on left trunk and extremities, and aberrant Mongolian spots on the upper back, lumbosacral area and buttocks. Due to clinical manifestation, the diagnosis of PPV was made. The patient was evaluated by Neurology, Traumatology and keeps on with ophthalmological controls. Complementary studies are important, to rule out extracutaneous manifestations in PPV. (Sociedad Argentina de Pediatría.)
Databáze:	MEDLINE
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