China
Africa
Explore chapters and articles related to this topic
Disorders of Keratinization and Other Genodermatoses
Published in Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang, Roxburgh's Common Skin Diseases, 2022
Roselyn Stanger, Nanette Silverberg
Differential diagnosis: Many of the skin diseases that present with erythroderma and ichthyosis can have overlapping clinical presentations. Genetic testing, with or without a skin biopsy, is often required to help make the diagnosis. The differential diagnosis can include congenital ichthyosiform erythroderma, Omenn syndrome, erythrokeratoderma variabilis, hyper-IgE syndrome, and psoriasis, among others.
Physiological and pathophysiological roles of hepoxilins and their analogs
Published in Drug Metabolism Reviews, 2023
Sara A. Helal, Fadumo Ahmed Isse, Samar H. Gerges, Ayman O. S. El-Kadi
The loss of function of these two genes is the second most common cause of a certain type of ichthyosis called non-bullous congenital ichthyosiform erythroderma (NCIE), which is a special form of autosomal recessive congenital ichthyoses (ARCIs) (Krieg et al. 2013). Interestingly, the deficiency in the HxA3 synthase enzyme in the skin leads to the formation of a novel form of ichthyosis, termed hepoxilin A3 synthase-linked ichthyosis (HXALI). The scales of the patients with HXALI showed high levels of 12 R-LOX and 12 R-HpETE and low levels of HxA3 which may be the cause for the mild expression of the disease in comparison to patients with NCIE (Nigam et al. 2008).
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
After the skin scaling was stopped, followed by full epithelialisation, further treatment with Emolliens ungventum, bathing in Exomega bath oil and the use of Aminoglycosides (gentamicin) ungventum in case of skin erosion were recommended. Gentamicin was used as an ungventum for external use to prevent skin infections most commonly caused by Staphylococcus bacteria. Upon the completion of the treatment in the duration of one month, the child was referred to a dermatologist who diagnosed Congenital ichthyosiform erythroderma (CIE), a variant of autosomal recessive congenital ichthyosis.