Epidermolytic Ichthyosis (Epidermolytic Hyperkeratosis or Bullous Congenital Ichthyosiform Erythroderma) Questions & Answers

Updated: Aug 23, 2019
  • Author: Brittany G Craiglow, MD; Chief Editor: Dirk M Elston, MD  more...
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Questions & Answers

Overview

What is epidermolytic ichthyosis (EI)?

What is the pathophysiology of epidermolytic ichthyosis (EI)?

What causes epidermolytic ichthyosis (EI)?

What is the incidence of epidermolytic ichthyosis (EI)?

What are the racial predilections of epidermolytic ichthyosis (EI)?

What are the sexual predilections of epidermolytic ichthyosis (EI)?

When is the onset of epidermolytic ichthyosis (EI)?

What is the prognosis of epidermolytic ichthyosis (EI)?

What is included in the patient education about epidermolytic ichthyosis (EI)?

Presentation

Which clinical history findings are characteristic of epidermolytic ichthyosis (EI)?

Which physical findings are characteristic of epidermolytic ichthyosis (EI)?

What are the possible complications of epidermolytic ichthyosis (EI)?

DDX

What are the differential diagnoses for Epidermolytic Ichthyosis (Epidermolytic Hyperkeratosis or Bullous Congenital Ichthyosiform Erythroderma)?

Workup

Which lab tests are performed in the workup of epidermolytic ichthyosis (EI)?

What is the role of genetic testing in the workup of epidermolytic ichthyosis (EI)?

What is the role of skin biopsy in the workup of epidermolytic ichthyosis (EI)?

How is epidermolytic ichthyosis (EI) diagnosed prenatally?

Which histologic findings are characteristic of epidermolytic ichthyosis (EI)?

Treatment

How is epidermolytic ichthyosis (EI) treated?

What is the role of surgery in the treatment of epidermolytic ichthyosis (EI)?

Which specialist consultations are beneficial to patients with epidermolytic ichthyosis (EI)?

Which dietary modifications are used in the treatment of epidermolytic ichthyosis (EI)?

Which activity modifications are used in the treatment of epidermolytic ichthyosis (EI)?

What is included in the long-term monitoring of epidermolytic ichthyosis (EI)?

Medications

What is the role of medications in the treatment of epidermolytic ichthyosis (EI)?