Ichthyosis hystrix Curth-Macklin type in an African girl

Ichthyosis hystrix Curth-Macklin type is a rare autosomal dominant skin disorder characterized by extensive hyperkeratosis and palmo-plantar keratoderma. It results from heterozygous frameshift mutation in keratin 1 gene (KRT1). Histological features, showing perinuclear vacuolization and binucleated cells, are similar to those of epidermolytic hyperkeratosis except for the absence of epidermolysis. The present report describes the condition in a 16-year-old African girl where available treatment was disappointing.