LETTERS TO THE EDITOR Phacomatosis Pigmentokeratotica Complicated with Juvenile Onset Hypertension

Sir, The association of a speckled lentiginous naevus, an organoid naevus with sebaceous differentiation and skeletal and neurological anomalies, constitutes a specific syndrome that has been called phacomatosis pigmentokeratotica (1). It has been hypothesized that the co-occurrence of the two different naevi reflects a so-called twin spot phenomenon (1 – 3). We herein describe an additional case with this rare condition, consisting of organoid epidermal naevus, speckled lentiginous naevus, microphthalmia, kyphoscoliosis, arachnoid cyst, solid tumours at the spinal roots and, in particular, juvenile hypertension.