A cross-sectional study on quality of life in adult indolent systemic mastocytosis and its association with cutaneous involvement

Abstract

Background

Systemic mastocytosis (SM) is characterized by abnormal mast cell accumulation in various tissues, including the skin, and encompasses several clinical variants, ranging from less aggressive to more severe forms. ISM, the most common variant, often presents with cutaneous manifestations, causing significant impairment in QoL due to mediator-release symptoms and stigma associated with typical cutaneous lesions.

Objectives

The study aimed to assess QoL impairment in ISM patients and its correlation with demographic and clinical data.

Methods

A cohort of 52 adult ISM patients was evaluated using the Dermatology Life Quality Index (DLQI) and the mastocytosis QoL questionnaire (MC-QoL). Questionnaire scores were then analyzed in relation to the clinical and demographic characteristics of the patients.

Results

Over half of the patients experienced mild or higher levels of impairment in QoL, with female patients showing greater impairment than males. Patients with recent symptom onset reported higher MC-QoL scores, possibly due to psychological factors and lack of disease knowledge. Cutaneous symptoms significantly impacted QoL, and the visibility of lesions affected DLQI scores more than MC-QoL scores. A moderately strong correlation was observed between DLQI and MC-QoL scores, particularly in the Skin and Social Life domains. The study highlights gender differences in QoL impairment, suggesting the need for further investigation into potential social or cultural factors.

Conclusions

While the study provides valuable insights into QoL impairment in ISM patients, its monocentric nature and small sample size are notable limitations. Further research is warranted to better understand the impact of SM on patients' well-being and to identify effective strategies for managing symptoms and improving overall QoL in this population.