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.
- Provider:StatPearls, LLC
- Activity Link: https://www.statpearls.com/ArticleLibrary/viewarticle/23337
- Start Date: 2023-09-01 05:00:00
- End Date: 2023-09-01 05:00:00
- Credit Details: AMA PRA Category 1 Credit™️: 1.0 hours
Nursing: 1.0 hours
Pharmacy: 1.0 hours - MOC Credit Details: ABS - 1.0 Point; Credit Type(s): Accredited CME (ABS)
ABPATH - 1.0 Point; Credit Type(s): Lifelong Learning (ABPATH)
ABIM - 1.0 Point; Credit Type(s): Medical Knowledge (ABIM)
ABS - 1.0 Point; Credit Type(s): Self-Assessment (ABS)
ABP - 1.0 Point; Credit Type(s): Lifelong Learning and Self-Assessment (ABP) - Commercial Support: No
- Activity Type: Enduring Material
- CME Finder Type: Online Learning
- Fee to Participate: Variable
- Measured Outcome: Learner Knowledge, Learner/Team Competence
- Provider Ship: Directly Provided
- Registration: Open to all
- Specialty: Cytopathology, Dermatopathology, Developmental-Behavioral Pediatrics, General Pediatrics, General Surgery, Hospital Medicine, Internal Medicine, Molecular Genetic Pathology, Neurodevelopmental Disabilities, Pediatric Emergency Medicine, Pediatric Endocrinology, Pediatric Pathology