Ichthyosis Hystrix. Presented by Francesco Ronchese, MD.
An 8-year-old boy has had an extensive dermatosis involving his entire skin except the face, palms, soles, dorsal distal phalanges, and nails since birth (Fig 1). His paternal grandfather died in 1965 at 58 of Hodgkin's disease. His father, 34, has the same condition as the son but his mother, 31, and a brother, 6, are normal.Examination reveals large scales separated by shallow fissures, giving the skin a reticulated appearance (Fig 2). The color is green-gray to almost black. The axillae, elbows, and knees show horny ridges (alligator skin). Some areas are free of scales and show erythema and bullae (Fig 3).Chromosomal analysis of 30 peripheral blood leukocytes revealed an apparently normal male karyotype (46,XY).
NEW ENGLAND DERMATOLOGICAL SOCIETY. Arch Dermatol. 1969;99(1):125-132. doi:10.1001/archderm.1969.01610190131025