erythrokeratodermia

Introduction

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
• widespread erythematous plaques, either stationary or migratory
• palmoplantar keratoderma
• erythematous & hyperkeratotic plaques (PSEK)

* images

Management

• keratolytics, emollients, calcipotriol, & retinoids useful
• oral retinoids usually achieve complete clearance of hyperkeratosis
  • they have limited efficacy for erythema
  • disease recurrence usually occurs on discontinuation

More general terms

• skin disease (dermatologic disorder, dermatopathy, dermatosis)

Database

• OMIM: https://mirror.omim.org/entry/602036