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

Clinical Practice

Erythrokeratoderma Online Journals

The clinically and genetically heterogeneous body of erythrokeratodermas encompasses different rare genetic skin disorders, additionally to autosomal dominant erythrokeratodermiavariabilis (EKV) and progressive symmetric erythrokeratoderma, also as autosomal dominant spin cerebellar ataxia with erythrokeratodermia. The skin lesions of EKV and PSEK show many resemblance, and a littlesubdivision of patients with features previously considered to be PSEK share the underlying explanation for EKV. Therefore, it had been recently proposed to classify this disorder as erythrokeratodermiavariabilitiesprogressiva (EKVP). Aside from , PSEK and overlapping sorts of erythrokeratoderma do exist as genetically distinct and heterogeneous entities from EKVP.Erythrokeratodermia variability’s (EKV) are a rare heterogeneous group of inherited cornification complications. They’recharacterized by two distinct morphological sorts of skin lesions: Accurate hyperkeratotic plaques and sharply marginated, pruritic, and migratory erythematous lesions. We report three cases of EKV alongside a review of literature.Erythrokeratodermas are rare group of disorders of keratinization. They’re classified into progressive symmetric erythrokeratoderma (PSEK) and erythrokeratodermia variabilis (EKV) with few individuals showing overlapping features of both conditions. EKV is inherited as an autosomal dominant disorder, rarely autosomal recessive pattern is reported in literature. Clinically, they manifest as migratory erythema, annular, or hyperkeratotic plaque. Atypical variants of EKV include erythema gyratumrepens like skin lesions, reticulateerythrokeratoderma, and “en cocardes” type.  

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