Acrokeratoelastoidosis (AKE) is a rare skin disorder characterized by a papular eruption and fragmentation of elastic tissue that primarily involves the margins of the hands and feet. The most common histopathologic findings of AKE are hyperkeratosis and degeneration of elastic fibers. Various treatment options have been tried with little help or without success. We report the third case of acrokeratoelastoidosis in an Arabic woman localized exclusively to the feet.
Download full-text PDF |
Source |
|---|
Publication Analysis
Top Keywords
Similar Publications
Asymptomatic pitted lesions of bilateral hands and feet.
JAAD Case Rep
December 2024
Department of Dermatology, SUNY Downstate Health Sciences University, Brooklyn, New York.
A mutation in CCDC91, Homo sapiens coiled-coil domain containing 91 protein, cause autosomal-dominant acrokeratoelastoidosis.
Eur J Hum Genet
June 2024
Shanghai Skin Disease Hospital, School of Medicine, Tongji Medical University, Shanghai, China.
Article Synopsis
- * Researchers analyzed a Chinese family with AKE symptoms using genome-wide linkage analysis and whole-exome sequencing, leading to the identification of a mutation in the CCDC91 gene which affects elastin transport.
- * Functional tests showed that a lack of CCDC91 leads to abnormal accumulation of proteins in skin cells, providing new insights into how this gene contributes to the disease's progression and advancing the understanding of AKE mechanisms.
"African" acral keratoderma: Further evidence for a single entity. A retrospective study of 42 patients.
Ann Dermatol Venereol
March 2023
Department of Dermatology, APHP, Saint-Louis Hospital, 75010 Paris, France.
Article Synopsis
- - The study explores various phenotypes of non-inflammatory palmar and plantar keratoderma (PPK) prevalent in Sub-Saharan African patients, proposing they might collectively be known as "African Acral Keratoderma" (AAK).
- - A review of 42 patients over 11 years revealed that most displayed mixed forms of keratoderma, with a significant proportion (40%) having familial histories, suggesting a potential genetic link.
- - Findings support the AAK concept, with the majority receiving topical treatments, and the rare condition "ainhum" managed surgically.
Acrokeratoelastoidosis: is there an association between asthma and sporadic cases in children?
Dermatol Online J
December 2020
Department of Dermatology, The University of Texas Health Science Center at Houston, Houston, TX.
Article Synopsis
- Acrokeratoelastoidosis (AKE) is a rare skin condition characterized by keratotic papules on the palms and soles, typically inherited in an autosomal dominant pattern, with some sporadic cases observed.
- A case involving an 11-year-old girl with asthma is discussed, along with a review of three similar cases in children, suggesting a potential link between AKE and asthma.
- Current research on AKE's causes and treatments is limited, emphasizing the need for further investigations to explore the relationship between AKE and asthma, which could lead to better treatment options.
Want 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!