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- Title
Phacomatosis pigmentokeratotica with nephroblastoma and juvenile hypertension.
- Authors
Jacobelli, Simon; Leclerc-Mercier, Stéphanie; Salomon, Rémi; Hartmann, Olivier; Brunelle, Frances; Happle, Rudolf; Bodemer, Christine; Hadj-Rabia, Smail
- Abstract
Phacomatosis pigmentokeratotica is characterized by the coexistence of an organoid epidermal naevus, follow-ing Blaschko's lines, and a large speckled lentiginous naevus, typically arranged in a chequerboard pattern. This entity has been isolated from the group of epidermal naevus syndromes and is frequently associated with extracutaneous anomalies. We report here the first observation of phacomatosis pigmentokeratotica associated with nephroblastoma. In addition to this paediatric renal tumour, the coexistence of juvenile arterial hypertension suggests an associated vascular defect. The link between the extracutaneous manifestations and cutaneous twin spot phenotype is discussed.
- Publication
Acta dermato-venereologica, 2010, Vol 90, Issue 3, p279
- ISSN
1651-2057
- Publication type
Journal Article
- DOI
10.2340/00015555-0824