Bullous congenital ichthyosiform erythroderma: safe and effective topical treatment with calcipotriol ointment in a child.

Sir, Bullous congenital ichthyosiform erythroderma (BCIE), or epidermolytic hyperkeratosis (MIM# 113.800), is a rare disorder of keratinization associated with blistering in its early phase. It was first described by Jean-Louis Brocq in 1902 as érythrodermie congénitale ichthyosiforme avec hyperépidermotrophie. Although most cases are sporadic, familial cases show an autosomal dominant pattern of inheritance. The disorder is linked to keratin clusters on chromosome 12q and 17q (1). Several researchers have described mutations in the highly conserved regions of keratins K1 and K10 (2, 3). Moreover, transgenic mice carrying mutant K10 have developed skin abnormalities similar to BCIE (4). Emollients are of only limited value in the treatment of BCIE, as the scales are often waxy and macerated (5). Retinoids, such as acitretin or etretinate administered systemically, are the treatment of choice in disorders of keratinization (6). However, they should be viewed with caution owing to their skeletal toxicity, especially in children and adolescents (7, 8). Effective therapies lacking significant adverse effects are therefore needed for these patients. Topical calcipotriol has been shown to be safe and effective in treating adolescent and adult patients with congenital ichthyoses (5) and other disorders of keratinization, including one case of BCIE (5). Here, we report the safe and successful long-term (w3 years) topical therapy with calcipotriol ointment in a 9-year-old boy with BCIE.