Hailey-Hailey disease (HHD), or familial benign chronic pemphigus, is characterized by a chronic relapsing course, with a difficult-to-treat condition that severely affects a patient’s quality of life. Although HHD is caused by a mutation of the ATP2C1 gene, there is no genotype–phenotype correlation according to clinical practice or literature reports. Patients usually present with clinical findings of HHD in apocrine gland-rich areas of the body after adolescence of life, severe in summer and mild even self-healing in winter. Treatments include antibiotics, anti-inflammatory, immunosuppression, and inhibition of sweat production by apocrine glands are partially effective. Based on such a distinct clinical phenotype, which is inseparable from season, location, sweat, and infection (bacteria), it appears that the environment should be the most important factor in the etiology of HHD. Therefore, this research aims to study the sweat gland function, sweat characteristics and the resulting underlying skin microecological changes of HHD patients in winter and summer, in order to explore effective, easy-to-operate and safe treatments and nursing methods, and reduce the current drug side effects and treatment-related complications. Single-cell sequencing of sweat gland cells and surrounding inflammatory infiltrating cells obtained in the biopsy is to understand the inflammatory and immune pathways of HHD. It is hoped that we will be able to lay down a foundation for further research on ATP2C1 gene and the mechanism of cell disconnection.
Audience Take Away Notes:
- It is a research about precious nursing. Precision nursing uses omic data within the context of lifestyle, social, economic, cultural and environmental influences to help individuals achieve well-being and optimal health.
- Nursing science could make contributions to precision health through symptom science on manifestation and management of symptoms of disease and treatment.
- Nurses have the potential to conduct nursing research that can identify interventions tailored to patients' unique omic, genetic/genomic, digital, lifestyle and environmental characteristics in order to promote optimal health outcomes.
- Nurses are well-positioned to lead the implementation of precision health through interprofessional collaboration, community outreach efforts, and coordination of care.