X-Linked Ichthyosis

Overview

4.4 out of 5 (5 Reviews)

Credits

1.00

Post Assessment Questions

8

Start Date

1 Jan 2021

Last Review Date

25 Jan 2024

Expiration Date

31 Dec 2023

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.

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.

Disclosures

The Campbell University Jerry M. Wallace School of Osteopathic Medicine (CUSOM) requires instructors, planners, managers, and other individuals who are in a position to control the content of this activity to disclose any real or apparent conflict of interest they or their immediate family may have as related to the content of this activity. All identified conflicts of interest are thoroughly vetted by CUSOM for resolution, to ensure fair balance, scientific objectivity of studies mentioned in the materials or used as the basis for content, and appropriateness of patient care recommendations.

CUSOM will identify, review, and resolve all conflicts of interest that faculty, authors, activity directors, planners, managers, peer reviewers, or relevant staff disclose prior to an educational activity being delivered to learners. Disclosure of a relationship is not intended to suggest or condone bias in any presentation but is made to provide participants with information that might be of potential importance to their evaluation of a presentation. Disclosure information for authors, editors, planners, peer reviewers, and/or relevant staff is provided with this activity.

Continuing Education Accreditation Information

 

 

The Campbell University Jerry M. Wallace School of Osteopathic Medicine (CUSOM) is accredited by the American Osteopathic Association to provide osteopathic continuing medical education for physicians. CUSOM designates this enduring material for a maximum of 1.00 AOA Category 1 B Credits and will report CME credits commensurate with the extent of the physician's participation in the activity.

Cancellation Policy: Cancellations must be received in writing and a money back guarantee is provided if not completely satisfied.

  • StatPearls and CUSOM reserve the right to cancel any course due to unforeseen circumstances. StatPearls and CUSOM will not be responsible for other expenses incurred by the participant in the unlikely event that the program is canceled.

Equal Opportunity

  • StatPearls and CUSOM are Equal Opportunity / Affirmative Action / Equal Access Institutions. 

Medium or Media Used:

  • Computer Requirements:  Internet Access
  • E-mail Address

Instructions for Credit

  1. Register for the activity and create a StatPearls login.     
  2. Review the required accreditation information:  Target audience, learning objectives and disclosure information.
  3. Complete the entire self-study activity.
  4. Complete the post-test assessments.
  5. Successfully pass the post-test with a minimum score of 100%.
  6. Complete the evaluation form.
  7. Obtain a certificate.

StatPearls and CUSOM adheres to AOA Standards regarding commercial support of continuing medical education. It is the policy of StatPearls and Campbell that the faculty and planning committee disclose real or apparent conflicts of interest relating to the topics of this educational activity, that relevant conflict(s) of interest are resolved and also that authors and editors will disclose any unlabeled/unapproved use of drug(s) or device(s) during their presentation. Detailed disclosure will be made prior to starting the activity.

The information provided at this CME/CE 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.

This course is intended for osteopathic physicians who wish to earn AOA CME credit. Take this version of the course to ensure you receive appropriate credit.

 

 
 

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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