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Retinoids in Keratinization Disorders
Published in Ayse Serap Karadag, Berna Aksoy, Lawrence Charles Parish, Retinoids in Dermatology, 2019
Ichthyosis disorders are characterized by dryness with marked desquamation. Several types of ichthyosis exist (1). Congenital ichthyosis requires lifelong treatment. Current evidence about ichthyosis treatments with oral retinoids including acitretin, isotretinoin and also oral liarozole as retinoic acid metabolism blocking agent is limited. Topical therapies, such as 5% urea, 5% lactic acid, 20% propylene glycol, calcipotriol, and liarozole 5% cream, have demonstrated some therapeutic efficacy in ichthyoses (2–19).
Ichthyotic disorders
Published in Biju Vasudevan, Rajesh Verma, Dermatological Emergencies, 2019
Many ichthyotic disorders can present as neonatal erythroderma and thus present as an emergency dermatological condition. Ichthyotic disorders presenting as erythroderma can be congenital in onset or can present in less than 1 month or greater than 1 month of life. Congenital ichthyosis is a group of hereditary disorders characterized by variable degrees of generalized, persistent scaling, with a chronic, lifelong course. Ichthyosis can be part of clinical syndromes with various systemic involvement. Nonsyndromic and syndromic ichthyosis can cause erythroderma in infants and children [1]. In a retrospective study of neonatal erythroderma conducted by Pruszkowski et al., ichthyosis was the cause in 24% of cases [2]. Sarkar et al. found ichthyosiform erythroderma to be the etiological diagnosis in 25% of neonatal erythroderma cases [3]. Different ichthyoses that can present as dermatological emergencies are denoted in Table 22.1.
Acitretin
Published in Sarah H. Wakelin, Howard I. Maibach, Clive B. Archer, Handbook of Systemic Drug Treatment in Dermatology, 2015
The licensed indications for acitretin are as follows: Severe extensive psoriasis that cannot be managed by topical treatment or phototherapy.Palmoplantar pustular psoriasis.Severe Darier’s disease.Severe congenital ichthyosis. Monotherapy is indicated for erythrodermic or pustular psoriasis while combination therapy (with phototherapy) is often used for chronic plaque psoriasis. The efficacy of acitretin monotherapy in chronic plaque psoriasis is limited and dose dependent, with approximately 70% of patients achieving a moderate or greater response. Various studies have reported partial clearance rates of 25–75% with daily doses of 30–40 mg. Lower doses (10 mg or 25 mg daily) have little therapeutic effect, whereas doses of 50 mg and 75 mg daily result in an improvement of at least 75% (PASI 75) in 25% of patients. Complete clearance is rare and adherence at high dosage is often limited by side-effects.
“Structural imprinting” of the cutaneous immune effector function
Published in Tissue Barriers, 2021
Yosuke Ishitsuka, Dennis R. Roop, Tatsuya Ogawa
The “thiol-based sensor–effector apparatus” the Kelch-like erythroid cell-derived protein with the cap-n-collar homology-associated protein 1 (KEAP1)/NF-E2-related factor 2 (NRF2) signaling pathway mechanistically revealed its own importance as a regulator of keratinization.10,11 Several mouse models have also shown that aberrant activation of the stress signaling pathway not only lead to autosomal recessive congenital ichthyosis (ARCI)-like hyperkeratosis,12,13 but also contributes to the pathogenesis of the most common skin disorders, such as atopic dermatitis (AD),14 psoriasis,15 or acne.16 In accordance with the “brick and mortar” model,4 the major later CE precursor LOR was dispensable for the lipid-based SC permeability barrier per se.4,17 Although somewhat anticipated from the structural basis,4 the discovery of the very mild LOR knockout (LKO) phenotype was counterintuitive and rather surprising.17,18 Nonetheless, LKO mice provided us opportunities to uncover the backup responses that maintain the quasi-homeostasis, both structurally and functionally.17,19–21
A review of IL-36: an emerging therapeutic target for inflammatory dermatoses
Published in Journal of Dermatological Treatment, 2022
Jonwei Hwang, Jonathan Rick, Jennifer Hsiao, Vivian Y. Shi
A study of ichthyoses included patients with congenital ichthyosiform erythroderma (n = 9), lamellar ichthyosis (n = 8), epidermolytic ichthyosis (n = 8), and Netherton syndrome (n = 4). Transepidermal water loss (TEWL) was correlated with increased serum IL-36α, β, and γ. Additionally, the inflammatory gene signature was mostly skewed toward IL-17/TNF-α coregulated genes, similar to psoriasis, which is known to have relationship with IL-36 (99). A study of a 51-year-old male with autosomal recessive congenital ichthyosis (ARCI) vs. 13 healthy controls also found increased upregulation of IL-36α and γ in patient skin (100).
Congenital ichthyosis (icthyosis lamellaris non bullosa) – therapy
Published in Journal of Obstetrics and Gynaecology, 2021
Miljana Z. Jovandaric, Svetlana J. Milenkovic
Appropriate eye care and pain control should be carried out for collodion babies with ectropion. Humidified incubators and water dressings followed by emollient agents are essential components of therapy. The congenital ichthyosis with large areas of skin erosions is always under the risk of heavy infections, even sepsis, therefore suitable topical and systemic antibacterial agents must be carefully identified and administered. Since there is no cure for ichthyosis, the treatment is aimed at the treatment of its signs and symptoms. The treatment may include creams, lotions or ointments to relieve dryness (Elias et al. 2012).