China
Africa
Explore chapters and articles related to this topic
Retinoids in Keratinization Disorders
Published in Ayse Serap Karadag, Berna Aksoy, Lawrence Charles Parish, Retinoids in Dermatology, 2019
Lamellar ichthyosis shows genetic heterogeneity. The disease usually appears at birth as the collodion baby. It affects both sexes equally, and prevalence of lamellar ichthyosis is less than 1/300,000. Hyperpyrexia and heat intolerance may be a problem in summertime or during exercise. Young children may have increased nutritional requirements due to their rapid growth and desquamation of their skin. Painful palmar and plantar fissures may be seen. Newborns are at risk for hypernatremic dehydration, secondary infection, and sepsis. The disorder persists throughout life.
Ichthyotic disorders
Published in Biju Vasudevan, Rajesh Verma, Dermatological Emergencies, 2019
Lamellar ichthyosis (LI) is a severe form of autosomal recessive congenital ichthyosis (ARCI). In the Asian population, LI is mainly caused by mutation in the TGM1 and ABCA12 genes. Clinically children present with collodion membrane at birth. After shedding of the collodion membrane, the skin is covered by large, thickened, quadrilateral, adherent lamellar scales all over the body, including the face, palms, and soles (Figure 22.3) [5]. There is diffuse hyperkeratosis with maceration and fissuring at flexural sites. Erythema is usually mild or absent. PPK is present in all cases of Lamellar ichthyosis (Figure 22.4) [9]. Ectropion is usually seen leading to dryness of the cornea and conjunctiva with exposure keratitis (Figure 22.5). Nails are thickened with subungual hyperkeratosis. Histology shows marked hyperkeratosis, hypergranulosis and moderate acanthosis, and few parakeratotic cells.
Principles of Clinical Diagnosis
Published in Susan Bayliss Mallory, Alanna Bree, Peggy Chern, Illustrated Manual of Pediatric Dermatology, 2005
Susan Bayliss Mallory, Alanna Bree, Peggy Chern
Differential diagnosisLamellar ichthyosisOther ichthyoses
Passive delivery of protein drugs through transdermal route
Published in Artificial Cells, Nanomedicine, and Biotechnology, 2018
Bivek Chaulagain, Ankit Jain, Ankita Tiwari, Amit Verma, Sanjay K. Jain
LPP examined in all species until now, has suggested that the presence of this phase plays an important role in skin barrier function. In the SC of patients suffering from several diseases characterized by very high skin permeability (atopic dermatitis, lamellar ichthyosis, psoriasis, Netherton syndrome and Chanarin–Dorfman disease), the periodicity of the LPP is decreased and its general appearance is significantly altered [18]. During investigations on the penetration of ethyl para-amino benzoic acid and benzoic acid across model SC membranes, the fluxes of these chemicals were found to elevate in the absence of LPP which supports the positive role of LPP in permeability across the skin. The lateral lipid organization within the lamellae strongly influences the SC permeability for water. The flux of water across human skin closely correlates with the type of solid phases present in the SC: the higher the OR content, the lower the flux of water [13,15,17]. The permeability through the skin therefore is the interplay of arrangement of lipids in lamellar and lateral layer. Absence of LPP favours high permeability whereas prevalence of OR arrangement in lateral layer reduces the permeation. Therefore, any approach for enhanced permeability through skin should be directed towards achieving LIQ arrangement of lipids laterally as opposed to OR and perturbing the LPP phase in lamellar symmetry.
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
Ichthyosis is a heterogeneous family of mostly genetic skin disorders. It includes a wide range of keratinising disorders with different aetiologies. The common feature is more or less generalised epidermal hyperkeratosis and dry, scaly skin. Depending on the type of ichthyosis and various individual and environmental factors, the severity of skin symptoms may range from mild xerosis, to massive hyperkeratosis and scaling all over the body, as in the case of lamellar ichthyosis (Metze et al. 2000).