Original articleMortality of patients with bullous pemphigoid in Korea
Section snippets
Study population
We retrospectively reviewed the medical records of all patients with BP who were diagnosed at the Gangnam Severance Hospital in Seoul, Korea, between January 1993 and March 2013. The following criteria were used to diagnose BP: (1) clinical suspicion of BP because of characteristic features; (2) histopathology consistent with BP (ie, subepidermal blisters with infiltration of inflammatory cells, particularly eosinophils); (3) direct immunofluorescence studies revealing linear C3 or/and
Demographic data and clinical features of patients
A total of 168 patients with BP who met the diagnostic criteria were identified, and 84 (50%) patients were female. The median age at the time of diagnosis was 73 years, and the mean age was 69.15 years (standard deviation, 15.70). The age range at the time of diagnosis was 6 to 99 years, and the majority of patients (96%) were >40 years of age. There were 54 (32.14%) patients with mild BP and 45 (26.79%) patients with severe BP (Table I). The median duration of follow-up for those alive at the
Discussion
Regarding the mortality of BP patients, conflicting results have been reported from different countries. In our study of 168 Korean patients with BP, the 1-year mortality rate was 19%, the 2-year mortality rate was 29%, and the 5-year mortality rate was 58%. These rates are lower than those reported in European studies.18, 19, 20 However, the mean age of the patients in our study was 69.15 years (SD, 15.70), which was approximately 10 years younger than that of previous European studies.
References (24)
- et al.
Pemphigoid diseases
Lancet
(2013) - et al.
Mortality rate of bullous pemphigoid in a US medical center
J Invest Dermatol
(2004) - et al.
Sepsis associated with dermatologic hospitalization is not the cause of high mortality of bullous pemphigoid in Europe
J Invest Dermatol
(2005) - et al.
Mortality of bullous pemphigoid: an evaluation of 223 patients and comparison with the mortality in the general population in the United States
J Am Acad Dermatol
(2008) - et al.
Incidence and mortality of bullous pemphigoid in France
J Invest Dermatol
(2012) - et al.
Bullous pemphigoid. Clinical and immunologic follow-up after successful therapy
Arch Dermatol
(1977) - et al.
Survival prognosis in pemphigoid. A cohort analysis of 78 patients
Ann Dermatol Venereol
(1995) - et al.
Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Bullous Diseases French Study Group
Arch Dermatol
(1995) - et al.
Anti-BP180 autoantibodies as a marker of poor prognosis in bullous pemphigoid: a cohort analysis of 94 elderly patients
Br J Dermatol
(1997) - et al.
High risk of death in elderly patients with extensive bullous pemphigoid
Arch Dermatol
(1998)
A comparison of oral and topical corticosteroids in patients with bullous pemphigoid
N Engl J Med
Risk factors for lethal outcome in patients with bullous pemphigoid: low serum albumin level, high dosage of glucocorticosteroids, and old age
Arch Dermatol
Cited by (45)
Explosive bullous pemphigoid with high serum total IgE: Serum IgE as a biomarker that reflects disease activity
2018, JAAD Case ReportsCitation Excerpt :Bullous pemphigoid (BP) is a chronic autoimmune blistering disease characterized by pruritic, tense bullae and urticarial plaques. BP is associated with significant mortality, with 1-, 2-, and 5-year mortality rates of 19.46%, 29.13%, and 58.03%, respectively, in South Korea.1 The main pathogenesis involves IgG autoantibodies against hemidesmosomal proteins: BP 180 and BP 230.
A Global View of Pemphigus: Geographical Variations
2024, Clinical Reviews in Allergy and ImmunologyAnalysis of the prognostic factors of bullous pemphigoid based on random forest model
2023, China Journal of Leprosy and Skin DiseasesMortality and prognostic factors in patients with bullous pemphigoid: a retrospective multicenter Italian study
2022, Journal of the European Academy of Dermatology and VenereologyAnalysis of prognostic factors of bullous pemphigoid
2022, China Journal of Leprosy and Skin Diseases
Funding sources: None.
Conflicts of interest: None declared.