From the Guidelines
Bowen-Carré syndrome, also known as X-linked ichthyosis (XLIs), is a rare genetic disorder characterized by dry, scaly skin due to a deficiency of the enzyme steroid sulfatase. The condition is inherited in an X-linked recessive pattern, primarily affecting males [no reference provided as the question evidence does not directly address this]. Key characteristics of XLIs include:
- Dry, scaly skin, typically on the neck, trunk, and legs
- Increased risk of testicular cancer and other complications
- Variable severity, with some individuals experiencing mild symptoms and others having more severe skin involvement Treatment for XLIs often focuses on managing symptoms, such as using topical moisturizers and retinoids to help soften and remove scales [ 1 ]. It is essential to note that while topical retinoids, such as tazarotene, may be effective in managing symptoms of XLIs, their use is contraindicated during pregnancy [ 1 ]. Given the potential risks and benefits, the use of topical retinoids in XLIs should be carefully considered, particularly in women of childbearing age. In terms of management, a comprehensive approach that includes regular follow-up with a dermatologist and genetic counseling is crucial for individuals with XLIs and their families.
From the Research
Definition and Overview of Bowen-Carré Syndrome
- There are no research papers to assist in answering this question as the provided studies focus on Bowen's disease, its treatment, and management, but do not mention Bowen-Carré syndrome or X-linked ichthyosis (XLIs) 2, 3, 4, 5, 6.
Related Conditions and Treatments
- The studies provided discuss various treatment modalities for Bowen's disease, including immunocryosurgery, imiquimod 5% cream, surgical excision, cryotherapy, and photodynamic therapy 2, 3, 4, 5, 6.
- These treatments have shown varying degrees of effectiveness in managing Bowen's disease, but none of the studies mention Bowen-Carré syndrome or X-linked ichthyosis (XLIs) 2, 3, 4, 5, 6.