Acta Derm Venereol 2004; 84: 397–416 LETTERS TO THE EDITOR Phacomatosis Pigmentokeratotica Complicated with Juvenile Onset Hypertension Etsuko Okada*, Atsushi Tamura and Osamu Ishikawa Department of Dermatology, Gunma University Graduate School of Medicine, 3-39-22 Showa-machi, Maebashi, Gunma 371-8511, Japan. *E-mail:
[email protected] Accepted January 20, 2004. Sir, left orbit. The result of an electroencephalogram was The association of a speckled lentiginous naevus, an normal. organoid naevus with sebaceous differentiation and We performed surgical resections of several skin skeletal and neurological anomalies, constitutes a lesions. Histological studies of the verrucous plaques of specific syndrome that has been called phacomatosis the face showed hyperkeratosis and papillomatous pigmentokeratotica (1). hyperplasia of the epidermis. Numerous mature seb- It has been hypothesized that the co-occurrence of aceous glands were present in the upper dermis. The the two different naevi reflects a so-called twin spot histological findings of the plaques of the head and phenomenon (1 – 3). We herein describe an additional back were almost the same as those of the face, being case with this rare condition, consisting of organoid consistent with naevus sebaceous. epidermal naevus, speckled lentiginous naevus, micro- phthalmia, kyphoscoliosis, arachnoid cyst, solid tumours at the spinal roots and, in particular, juvenile DISCUSSION hypertension. The epidermal naevus syndromes include different diseases that have the common feature of mosaicism. Among these phenotypes, the Proteus, CHILD, naevus CASE REPORT comedonicus, Becker naevus and Schimmelpenning syndromes have been delineated (2). Happle et al. (1) An 18-year-old Japanese man presented with verrucous proposed phacomatosis pigmentokeratotica as the plaques on his head, which had been noted at birth and name for a distinct type of epidermal naevus syndrome became verrucous at puberty.