ISSN: 2044-9038 (Print) 2044-9046 (Electronic)

Clinical Practice

Open Access Articles On Erythrokeratoderma

 This is due to several genetically inherited disorders, mostly autosomal dominant. This means that the gene comes from one parent and that an individual with the disease may pass it on to 50% of his or her children. Sporadic cases occur due to new genetic mutations at conception. Erythrokeratoderma is diagnosed by its clinical appearance. A skin biopsy can be performed for histology, but there are no distinctive features. In most types of erythrokeratoderma, the underlying defect appears to be a mutation in one of the connexin genes. Connexins are gap junction proteins, found in the channels that connect adjacent cells. Different connexins are found in different tissues, accounting for variability in presentation. Genetic counselling should be offered to affected individuals and their families of childbearing age. In time, genetic tests for the specific disorders may be available to some families. There is no specific or curative treatment for erythrokeratoderma. Minimising temperature changes and mechanical friction is important. Symptomatic improvement can be obtained by: Emollients Keratolytics such as urea, salicylic acid or alpha hydroxy acids Topical steroids Topical retinoids Oral retinoids such as acitretin or isotretinoin. These thin down the plaques and reduce scaling but the redness persists. It can be difficult to decide whether to take them longterm for erythrokeratoderma, because they may cause some adverse effects. Because erythrokeratoderma is rare, the classification of the different types is still evolving. There are a few well-defined syndromes 

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