Family impact of pemphigus disease in an Iranian population using the Family Dermatology Life Quality Index

Abstract

Background Pemphigus vulgaris (PV) is a rare but seriously disabling disorder of the skin and mucous membranes that can gravely impact the quality of life (QoL) of patients. Objective The aim of the present study was to identify how family members of patients with PV are affected by the disease. Methods A total of 118 patients with confirmed PV and one of their family members (caregivers) were enrolled in the study. To calculate disease severity, the Autoimmune Bullous Skin Disorder Intensity Score was used. The Persian version of the Dermatology Life Quality Index (DLQI) questionnaire was used to evaluate the QoL of patients and the Family Dermatology Life Quality Index (FDLQI) to evaluate the QoL of caregivers. Results The mean age of patients was 43.14 ± 12.5 years. Ninety patients (76.3%) were female. Eighty-one patients (68.6%) had the mucocutaneous phenotype and 37 cases (31.4%) the mucosal phenotype. The DLQI score was 10.1 ± 7.1 for patients. The DLQI score was higher for patients with the mucocutaneous phenotype (11.8 ± 7.5) than those with the mucosal phenotype (6.4 ± 4.9; p < .001). QoL was significantly affected by disease severity. FDLQI score was 13 ± 7 for caregivers, and was significantly higher in older caregivers and married ones. There was a positive correlation between patients’ admission frequencies and FDLQI score. FDLQI score was also significantly affected by the Autoimmune Bullous Skin Disorder Intensity Score of patients’ disease severity. The QoL of patients and their caregivers showed a significant positive correlation. Conclusion The QoL of patients and their families are impaired significantly, and is considerably prominent in the mucocutaneous phenotype of PV and more severe forms. Limitation Pemphigus Disease Area Index (PDAI) and Autoimmune Bullous Disease QoL (ABQoL) were not used in this study.

Impairment of sexual function in patients with pemphigus

“…A cohort study from Iran of 118 PV patients found a lower effect on sexual relationships as reported in the DLQI than other aspects of the DLQI. 2 However, this study had no control group. In another small study presented at the AAD-VMX, significantly reduced sexual function was reported in patients with PV.…”

Burden of pemphigus vulgaris with a particular focus on women: A review

“…Accordingly, pemphigus caused a remarkable impairment in the quality of life (QoL) of patients' families, associated with disease severity, mucocutaneous involvement, shorter disease duration, and male sex of patients that worsened by older age, low education, and the marital status of caregivers. 104,105 Improving knowledge and problem-solving abilities in patients and families can greatly reduce the disease burden. 106 QoL instruments and measurements Several assessment tools have been utilized to evaluate the QoL of patients with PV.…”

Exploring the Experiences of Patients with Autoimmune Skin Diseases During the COVID-19 Pandemic

“…5 Pemphigus vulgaris is one of the most serious and dangerous of these diseases and usually leads to hospitalization. 6,7 In addition to increased risk from the use of immunosuppressive medications, patients with autoimmune skin diseases are at a higher risk of infection than the general population because of their compromised skin integrity (eg, blisters, scratching). This increased risk led to concerns during the COVID-19 pandemic, especially when other predisposing factors were present.…”