Do people with ichthyosis have long lives?

From the Guidelines

People with ichthyosis can generally have normal lifespans, though this depends on the specific type they have, and there is no direct information available on "ichthymus" as it seems to be a misspelling or variation of the term "ichthyosis". The management of congenital ichthyoses is crucial for improving the quality of life, as these conditions can have a major effect on patients' quality of life and require lifelong treatment 1. Most common forms like ichthyosis vulgaris and X-linked ichthyosis are compatible with normal life expectancy, requiring only management of symptoms through regular moisturizing, keratolytic agents (like salicylic acid or urea-containing creams), and sometimes oral retinoids for severe cases. However, certain rare and severe forms, such as Harlequin ichthyosis, can be life-threatening, especially in infancy, though improved treatments have increased survival rates.

Key Considerations

• The condition is caused by genetic mutations affecting skin cell turnover or barrier function, resulting in the characteristic scaling and dryness, but these skin symptoms typically don't directly impact longevity in most cases.
• People with ichthyosis face challenges primarily related to skin care, infection prevention, temperature regulation, and sometimes associated conditions depending on the specific type.
• European guidelines for the management of congenital ichthyoses have been developed, covering topical therapies, systemic therapies, psychosocial management, communicating the diagnosis, and genetic counseling 1.

Treatment and Management

• Regular moisturizing and the use of keratolytic agents are essential for managing symptoms.
• Oral retinoids may be used for severe cases.
• Psychosocial management and genetic counseling are also important aspects of care. Given the lack of direct information on "ichthymus" and assuming it refers to "ichthyosis", the most appropriate approach is to consider the specific type of ichthyosis and its associated challenges and management options to determine the potential impact on lifespan.

From the Research

Ichthyosis and Life Expectancy

• There is no direct evidence to suggest that people with ichthyosis have longer lives 2, 3, 4.
• The available studies focus on the quality of life and health-related quality of life among patients with ichthyosis, rather than life expectancy 2, 3, 4.
• Ichthyosis is a heterogeneous group of skin diseases that can have a significant impact on a person's quality of life, with many patients reporting physical problems and impaired mobility 2, 3.

Quality of Life and Ichthyosis

• Studies have shown that ichthyosis can have a negative impact on various aspects of life, including childhood and adulthood experiences 3.
• The Nottingham Health Profile (NHP) questionnaire and other tools have been used to investigate quality of life in patients with ichthyosis, with results indicating a significant impairment in quality of life compared to the general population 2, 3, 4.
• Coping strategies used by patients with ichthyosis, such as hiding of the skin and developing shyness, can also affect their quality of life 3.

Treatment and Management of Ichthyosis

• Systemic retinoids have been used in the treatment of ichthyosis and related skin types, with careful surveillance and management of side effects being crucial 5, 6.
• The use of retinoids in children and adolescents requires consideration of potential side effects, including bone and eye effects, as well as contraceptive concerns 6.