International Jtjiirnal of Dermatology, Vol. 35, No. 9, ScptL*mbcr 11. Coskey RJ, Mehregan A. Papular mucinosis. Int J Der- 14. matol 1977; 16:741-744. 12. Harris RB, Perry HO, Kyle RA, et al. Treatment of scle- romyxedema with melphalan. Arch Dermatol 1979; 115:295-299. 15. 13. Rufz-Rodriguez R, Maurer TA, Berger TG. Papular mu- cinosis and human immunodeficiency virus infection. Arch Dermatol 1992; 128:995-996. Smith KJ, Skelton HG, Jame W, et al. Papular eruption of human immunodeficiency virus disease. A review of the clinical, histologic and immunohistochemical findings in 48 cases. Am J Dermatopathol 1991; 13:445-451. Perrin C, Durant JM, Lacour JP, et al. Horny perifollic- ular mucinosis. An atypical pityriasis rubra pilaris-like eruption associated with HIV infection. Am J Der- matopathol 1993; 15:358-362. : •" CAMEO LINEAR AND WHORLED NEVOID HYPERMELANOSIS IN A BOY WITH MENTAL RETARDATION AND CONGENITAL DEFECTS CARMELO SCHEPIS, M.D., MADDAt.ENA SIRAGUSA, M.D., ANTONINO ALBERTI, M.D., AND VITTORIO CAVALLARI, M.D. A 12-year-old boy with severe mental retardation, had macrocephaly with hypertelorism, epicanthus, a bifid uvula, pectus excavatum, asymmetry of the extremities, and hy- potrophy of the legs. He had also scoliosis and a supinated varus foot. Eye examination showed horizontal nystagmus and exotropia of the right eye. Auditory evoked potentials in- dicated a transmission-type deficit. Results of routine blood tests, electroencephalogram, and karyotyping were normal. A cerebral computer tomogram scan showed dilation of the lateral ventricles without signs of increased intracranial pres- sure. On examination of the skin, there were thin hyperchro- matic "V"-shaped streaks (Fig. 1) on the back, along Blaschko's lines that continued towards his flanks, especially on the left side. The streaks appeared reticulated arising from the coalescence of brown macules of a few millimeters in di- ameter (Fig. 2). Similar linear symmetrical lesions were also present along the flexor area of the upper extremities. The hyperpigmentation seemed to have developed during the perinatal period. The boy's palms and soles were spared. He had no particular dental anomalies. Skin biopsy specimens taken from normally pigmented areas as well as from adja- cent linear hyperpigmented zones, were processed for light and electron-microscopy. The morphologic analysis with light microscopy revealed prominent melanocytes in both specimens as well as an increase of melanin in the epider- mis. In the skin, appearing normal macroscopically, melano- From the Unit of Dermatology, Department of Pediatrics, Oasi Institute for Research on Mental Retardation and Brain Aging (IRCCS), Troina, Italy, and the Department of Human Pathology, University of Messina, Messina, Italy. Address for correspondence: Carmelo Schepis, M.D., Unit of Dermatology, Oasi Institute, Via Conte Ruggero, 73, 1-94018 Troina, Italy. cytes were prominent and widely distributed. Electron mi- croscopy showed melanocytes that exhibited dispersed chromatin, abundant rough endoplasmic reticulum, well developed melanosomes, and bundles of intermediate fila- ments of stage III or IV. Keratinocytes appeared well pre- Figure 1. Thin hyperchromic "V"-shaped streaks on the back. 654