Congenital Ichthyosiform Erythroderma Superimposed with Chronic Dermatophytosis: A Report of Three Siblings

Abstract

Congenital ichthyosiform erythroderma is an autosomal recessive ichthyosis characterized by severe scaling and erythroderma. We report a family of three siblings who were all born with a collodion membrane and presented with diffuse scaling and pruritus. All three children subsequently developed chronic cutaneous dermatophyte infections requiring oral antifungals. One child developed superinfection with methicillin-resistant Staphylococcus aureus requiring antibiotics.

An IL-17–dominant immune profile is shared across the major orphan forms of ichthyosis

“…Based on case reports and our experience, Staphylococcus aureus, dermatophyte, and candidal infections occur not infrequently in patients with ichthyosis, although S aureus infections are less common than in those with moderate-to-severe AD. 99,[112][113][114][115][116] Even with an anti-IL-17 drug (secukinumab) for psoriasis, only about 1% of patients have mild-to-moderate mucocutaneous infections, predominantly candidal. 117,118 The reactive role of T H 17/IL-23 skewing to the barrier defects versus a primary pathogenic role in patients with ichthyosis can only be clarified through future studies with targeted antagonists, which include careful monitoring for mucocutaneous infections.…”

Management of congenital ichthyoses: European guidelines of care, part two

“…Some forms of CI seem to be more prone to develop recurrent skins infections, including ARCIs, notably HI, epidermolytic ichthyosis (EI), Netherton syndrome (NS) and keratitis–ichthyosis–deafness (KID) syndrome . Many patients with ARCIs or KID syndrome experience recurrent dermatophytosis (e.g. from Trichophyton rubrum ).…”

Inherited ichthyosis and fungal infection: an update on pathogenesis and treatment strategies

“…Kamalam et al. described tinea glabrosa and tinea manuum and unguium (onychomycosis) associated with T. rubrum in two infants with RXLI and IV, respectively [9], while CIE with T. rubrum was also frequently reported [3, 10–12]. In addition to T. rubrum , T. mentagrophytes , Epidermophyton (E.) floccosum , T. tonsurans , T. verrucosum , Geomyces pannorum , and Candida (C.) albicans infections have also been recorded in patients with ichthyosis [6, 7, 13–16].…”

“…In addition to T. rubrum , T. mentagrophytes , Epidermophyton (E.) floccosum , T. tonsurans , T. verrucosum , Geomyces pannorum , and Candida (C.) albicans infections have also been recorded in patients with ichthyosis [6, 7, 13–16]. Some patients with ichthyoses combined with extensive and recalcitrant fungal infections have also been identified [3, 7, 11, 13, 17, 18]. Advances in technology have increased research into the mechanisms of fungal infection in ichthyosis patients [19] and have suggested a correlation between genotype and susceptibility to fungal infection [20].…”

“…They are divided into nonsyndromic and syndromic forms, both of which include many subtypes associated with mutations in different causative genes [1]. Although fungal infections are not uncommon, patients with ichthyoses seem to be at increased risk of developing cutaneous fungal infections [2, 3]. Chronic fungal infection is a common complication of the syndromic ichthyosis known as keratitis, ichthyosis and deafness (KID) syndrome [4].…”