Topical Tazarotene 0.05% versus Glycolic Acid 70% Treatment in X-linked Ichthyosis due to Extensive Deletion of the STS Gene

Abstract

Sir, X-linked ichthyosis (XLI) appears in the immediate neonatal period as a generalized large, slightly adherent, lightly coloured desquamation commonly affecting the scalp, preauricular area and posterior neck (1). In time, large, often dark scales, preferentially located on the trunk and extensor aspect of the extremities, become prominent. The palms, soles and face are generally spared (1). Cutaneous histopathological findings of XLI are not diagnostic, being characterized by orthokeratotic hyperkeratosis and a normal or slightly thickened granular layer. In the dermis, oedema and a slight perivascular inflammatory infiltrate can be observed (1). The most common associated extracutaneous findings include corneal opacities, cryptorchidism, epilepsy and electro-encephalographic changes (1). XLI is caused by deficient activity of the steroid sulphatase (STS) enzyme due, in most patients, to complete or partial deletions of the STS gene mapped on Xp22.3 (2-5). Therapeutic approaches for the treatment of mild to moderate cutaneous manifestations of XLI include keratolytic agents, moisturizers and topical retinoids, while systemic retinoids have been successfully employed in severe forms (6, 7). We describe a 22-year-old man with XLI associated with epilepsy. A within-patient study comparing topical tazarotene 0.05% and glycolic acid 70%

Evidence of the high prevalence of neurological disorders in nonsyndromic X-linked recessive ichthyosis: a retrospective case series

“…Cognitive and behavioural disorders, including ADHD (up to 40% of patients in some series), autism (up to 20%), epilepsy and cognitive impairment, have also been reported in patients with nonsyndromic XLI . In our series, nine of 30 patients (30%) had ADHD and four of 30 (13%) suffered epilepsy, but none showed autism or cognitive impairment.…”

Perspective and Consensus Opinion: Good Practices for Using Organotypic Skin and Epidermal Equivalents in Experimental Dermatology Research

“…and reversing ectropion in congenital ichthyosis. 9,10,18,20,21,24,[48][49][50][51] Most case reports have focused on lamellar ichthyosis, although improvement also has been reported for other forms of ichthyosis, including other autosomal recessive congenital ichthyoses, recessive X-linked ichthyosis, 52 epidermolytic ichthyosis, 53,54 and erythrokeratodermia variabilis. 55 There are more reports on the effective use of tazarotene than other topical retinoids, but no direct comparisons have been performed.…”

Ichthyosis: presentation and management

“…Evidence supporting their use mainly derives from small clinical studies, but expert consensus guidelines have been published [28,29]. Tazarotene is available in North America (not Europe) for off-label treatment [30][31][32][33][34][35]. Tazarotene also encourages contracture release and reverses ectropion [32,33].…”