Contents

Search

erythrokeratodermia

A group of disorders Genetics: - may be family history - defects in LOR are a cause of progressive symmetric erythrokeratodermia (PSEK) Clinical manifestations: - may begin in childhood as diffuse, symmetrically distributed migratory erythematous plaques that gradually spread centrifugally, forming large figurate lesions that later become fixed [2] - widespread erythematous plaques, either stationary or migratory - palmoplantar keratoderma - erythematous & hyperkeratotic plaques (PSEK) * images [2] Management: - keratolytics, emollients, calcipotriol, & retinoids useful [2] - oral retinoids usually achieve complete clearance of hyperkeratosis - they have limited efficacy for erythema - disease recurrence usually occurs on discontinuation [2]

General

skin disease (dermatologic disorder, dermatopathy, dermatosis)

Database Correlations

OMIM 602036

References

  1. UniProt :accession P23490
  2. Rozas-Munoz E, Ramos-Arancibiac N, Madariaga JA. Erythrokeratodermia. JAMA Dermatol. 2023;159(8):875-876. PMID: 37285148 https://jamanetwork.com/journals/jamadermatology/fullarticle/2805525