X-Linked Ichthyosis

Overview

4.4 out of 5 (5 Reviews)

Credits

1.00

Pharmacology Credits

0.5

Post Assessment Questions

8

Start Date

1 Sep 2023

Last Review Date

25 Jan 2024

Expiration Date

31 Aug 2026

Estimated Time To Finish

60 Minutes


 
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Activity Description

X-linked ichthyosis, also known as steroid sulfatase (STS) deficiency and X-linked recessive ichthyosis, is a recessive, nonsyndromic genetic skin disorder. This condition is caused by a mutation or deletion in the STS gene, which is responsible for encoding the steroid sulfatase enzyme, causing the complete loss of steroid sulfatase enzyme activity. Although X-linked ichthyosis equally affects all ethnic groups and races worldwide, the condition predominantly affects males rather than females, as suggested by its name. Affected patients can normally produce skin cells but cannot shed them correctly, leading to dry skin that accumulates in the form of polygonal scales. The extracutaneous features include asymptomatic punctate corneal opacities, cryptorchidism, and cognitive or behavioral disorders, such as attention-deficit hyperactivity disorder.

The deficiency of steroid sulfatase results in the accumulation of cholesterol sulfate and depletion of cholesterol levels, leading to an abnormal skin barrier and retention of corneocytes. Although definitive treatment does not exist for X-linked ichthyosis, various options are available for long-term management. The primary treatment objectives involve reducing dryness, minimizing scale formation, and enhancing skin appearance without causing irritation. This activity comprehensively reviews the pathophysiology, diagnosis, and management of X-linked ichthyosis, equipping healthcare professionals with the essential knowledge to deliver optimal, patient-centered care to individuals affected by X-linked ichthyosis.


Target Audience

This activity has been designed to meet the educational needs of physicians, physician associates, nurses, pharmacists, and nurse practitioners.

Learning Objectives

At the conclusion of this activity, the learner will be better able to:

  • Identify the characteristic features of X-linked ichthyosis, including thick, dry, scaling skin, and recognize extracutaneous manifestations such as asymptomatic punctate corneal opacities, cryptorchidism, and cognitive or behavioral disorders.

  • Apply knowledge of the genetic underpinnings of X-linked ichthyosis, particularly the STS gene mutation or deletion, to inform accurate diagnoses and genetic counseling for affected individuals and their families.

  • Select appropriate interventions for long-term management and evidence-based treatment strategies for X-linked ichthyosis, considering individual patient preferences and characteristics to optimize treatment adherence.

  • Coordinate care efforts among interprofessional healthcare providers from dermatology, ophthalmology, psychology, and genetics to facilitate seamless communication, patient education, and ongoing monitoring to enhance overall outcomes for individuals affected by X-linked ichthyosis.

  • Outline the pharmacologic therapy as it applies to X-Linked Ichthyosis

Disclosures

StatPearls, LLC requires everyone who influences the content of an educational activity to disclose relevant financial relationships with ineligible companies that have occurred within the past 24 months. Ineligible companies are organizations whose primary business is producing, marketing, selling, re-selling, or distributing healthcare products used by or on patients. All relevant conflict(s) of interest have been mitigated. Hover over contributor names for financial disclosures. Others involved in planning this educational activity have no relevant financial relationships to disclose.

Commercial Support: This activity has received NO commercial support.

Continuing Education Accreditation Information

In support of improving patient care, StatPearls, LLC is jointly accredited by the Accreditation Council for Continuing Medical Education (ACCME), the Accreditation Council for Pharmacy Education (ACPE), and the American Nurses Credentialing Center (ANCC) to provide continuing education for the healthcare team.

 

Nurse Practitioners: StatPearls designates this activity for 1.00 ANCC contact hour(s), which includes 0.5 hour(s) of pharmacology. NPs should only claim credit commensurate with the extent of their participation in the activity.

Please consult your professional licensing board for information on the applicability and acceptance of continuing education credit for this activity.

Method of Participation and Credit

  1. Register for the activity.    
  2. Review the target audience, learning objectives, and disclosure information.
  3. Study the educational content of the enduring material.
  4. Choose the best answer to each activity test question. To receive credit and a certificate, you must pass the test questions with a minimum score of 100%.
  5. Complete the post-activity assessment survey.

If you have concerns regarding the CE/CME system, please contact support@statpearls.com.

Disclaimer

This educational activity was planned and produced in accordance with the ACCME Standards for Integrity and Independence in Accredited Continuing Education. Faculty may discuss investigational products or off-label uses of products regulated by the FDA. Readers should verify all information before employing any therapies described in this educational activity.

The information provided for this activity is for continuing education purposes only and is not meant to substitute for the independent medical/clinical judgment of a healthcare provider relative to diagnostic and treatment options of a specific patient’s medical condition. The information presented does not necessarily reflect the views of StatPearls or any commercial supporters of educational activities on statpearls.com. StatPearls expressly disclaims responsibility for any adverse consequences resulting directly or indirectly from information in the course, for undetected error, or through a participant's misunderstanding of the content.

Unapproved Uses of Drugs/Devices: In accordance with FDA requirements, the audience is advised that information presented in this continuing education activity may contain references to unlabeled or unapproved uses of drugs or devices. Please refer to the FDA-approved package insert for each drug/device for full prescribing/utilization information.

Cancellation Policy: Please see the cancellation policy. StatPearls, LLC reserves the right to cancel any course due to unforeseen circumstances.

 

 
 

Reviews

Mani Kiran Reddy Y. on 12/15/2021

Dr.Mallikarjun K. on 4/20/2022

MANJARI G. on 8/15/2022

Jerin M. on 2/29/2024

Sam A. on 4/7/2024

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