Erythrokeratoderma variabilis, also known as Mendes da Costa syndrome, is a genodermatosis belonging to the group of diseases known as the erythrokeratodermias. Erythrokeratoderma variabilis is characterized by two distinctive manifestations: well-demarcated, variable, transient, figurate patches of erythema, and localized or generalized hyperkeratotic plaques. Treatments include topical retinoic acid, salicylic acid, and alpha-hydroxy acid in petrolatum, but all have been reported to have limited, variable success rates. We report a child with erythrokeratoderma variabilis with no family history of this entity, successfully treated with topical tazarotene.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1111/j.1525-1470.2006.00252.x | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Four cases of Chanarin-Dorfman syndrome presenting with different types of erythrokeratoderma.
Pediatr Dermatol
November 2024
Department of Dermatology-Venereology and Allergology, Universitätsklinikum Schleswig-Holstein, Kiel, Germany.
Chanarin-Dorfman syndrome (CDS) is a multisystem autosomal recessive disorder due to variants of the ABHD5 gene, characterized by lipid vacuoles in the liver and leukocytes, and possible involvement of eyes, ears, skeletal muscle, and central nervous system. CDS may present with skin changes, most commonly congenital non- bullous ichthyosiform erythroderma, however erythrokeratoderma-like findings have been rarely reported in CDS patients. Herein, we report clinical, histopathological and genetic findings of four patients with CDS presenting with different clinical forms of erythrokeratoderma (three with progressive symmetric erythrokeratoderma-like features and one with erythrokeratoderma variabilis (EKV)-like features), including one patient with a novel mutation in ABHD5.
View Article and Find Full Text PDFAlitretinoin as a Treatment Modality for Ichthyosis in Women of Childbearing Age: A Case Series and Review of the Literature.
Dermatology
February 2024
Department of Dermatology, Maastricht University Medical Centre +, Maastricht, The Netherlands.
Article Synopsis
- Acitretin, a common oral treatment for ichthyosis, requires extended contraceptive measures post-treatment, posing challenges for women of childbearing age; alitretinoin is suggested as a safer alternative that allows pregnancy one month after stopping the medication.
- A study involved nine women aged 19-31 with various ichthyosis types, treated with alitretinoin for 2-28 months, showing significant improvements in skin severity scores.
- Side effects were generally mild and reversible; literature search revealed that most previous studies on alitretinoin for ichthyosis also reported significant improvements, indicating its potential as a valuable treatment option.
An uncommon form of ichthyosiform erythroderma, Netherton Syndrome (NS) is inherited by an autosomal recessive pattern. Owing to eczematous skin lesions and the clinical features of atopy, NS is often initially diagnosed as atopic dermatitis. There are very few reports on NS in India.
View Article and Find Full Text PDFErythrokeratoderma Variabilis (EKV) is a rare genodermatosis, characterized by variable erythematous and hyperkeratotic skin lesions. It is most often transmitted in autosomal dominant manner (AD). Casual mutations were found in the and genes encoding connexins 31 and 30.
View Article and Find Full Text PDFFormation of keto-type ceramides in palmoplantar keratoderma based on biallelic KDSR mutations in patients.
Hum Mol Genet
March 2022
Lipid Pathobiochemistry Group, German Cancer Research Center (DKFZ), 69120 Heidelberg, Germany.
Functional skin barrier requires sphingolipid homeostasis; 3-ketodihydrosphingosine reductase or KDSR is a key enzyme of sphingolipid anabolism catalyzing the reduction of 3-ketodihydrosphingosine to sphinganine. Biallelic mutations in the KDSR gene may cause erythrokeratoderma variabilis et progressive-4, later specified as PERIOPTER syndrome, emphasizing a characteristic periorifical and ptychotropic erythrokeratoderma. We report another patient with compound heterozygous mutations in KDSR, born with generalized harlequin ichthyosis, which progressed into palmoplantar keratoderma.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!