Prevalence, Spectrum and Clinical Implications of Malignancies in Patients with Bullous Pemphigoid

Abstract

Current research on the malignancy rate and spectrum of malignancies in patients with bullous pemphigoid is contradictory. The aims of this study were to determine the prevalence and spectrum of malignancy in patients with bullous pemphigoid and to compare demographic, clinical, therapeutic and outcome data between bullous pemphigoid patients with and without malignancy. This retrospective cohort study enrolled 335 patients (194 women and 141 men; mean age at diagnosis of bullous pemphigoid 77.5 ± 12 years) followed up at an Israeli tertiary centre between January 2009 and December 2019: 107 (32%) had malignancy and 228 (68%) did not. Malignancy occurred before and after bullous pemphigoid diagnosis in 82 (77%) and 25 (23%) patients, respectively. Bullous pemphigoid patients with cancer were older (p = 0.02) and had a higher mortality rate (p < 0.0001) than those without malignancy. The 2 groups did not differ in terms of sex, comorbidities, or clinical characteristics. Those who developed malignancy before bullous pemphigoid were younger than those who developed malignancy after bullous pemphigoid (mean age 69.3 vs 82.4 years, p < 0.0001). Overall malignancy rates did not differ between patients with bullous pemphigoid and the general population; therefore, comprehensive malignancy workup may be unnecessary. However, patients with bullous pemphigoid had a greater risk of melanoma (10.7% vs 4.3%, p = 0.0005); therefore, routine skin screening may be recommended.

Relationship between bullous pemphigoid and malignancy: A Mendelian randomization study

“…This conflicts with the conclusions of our study. For example, Baum et al 30 reported a positive correlation between BP and an increased susceptibility to melanoma. Schulze et al 6 noted that patients with BP exhibited an increased risk of hematological malignancies (OR 2.55, 95% CI 2.07-3.13).…”

“…This conflicts with the conclusions of our study. For example, Baum et al 30 . reported a positive correlation between BP and an increased susceptibility to melanoma.…”

Advancements in Bullous Pemphigoid Treatment: A Comprehensive Pipeline Update

“…In a retrospective cohort study involving 335 BP patients there was no significant difference in malignancy rates between BP patients and the general population [11]. Consequently, a comprehensive malignancy work-up might not be necessary for BP patients However, the study revealed that BP patients faced a higher risk of melanoma (10.7% vs. 4.3%, p < 0.001), indicating the potential benefit of routine skin screenings [11].…”

“…Among these patients, 127 (87.0%) attained disease control within 4 weeks, defined as the absence of new lesions and itching, as well as the healing of existing lesions [69]. The median (interquartile range [IQR]) time for achieving this control was 14 (7)(8)(9)(10)(11)(12)(13)(14) days [69]. Additionally, 52 (35.6%) patients experienced complete remission, while 13 (8.9%) BP patients relapsed during the observation period [69].…”