Is transition between subtypes of pemphigus possible? A series of pemphigus vulgaris patients showing the transition to pemphigus foliaceus

Abstract Background Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are subtypes of pemphigus with distinct clinical and laboratory features. The transition between these two subtypes has rarely been reported previously. Methods The data of PV patients who exhibited clinical and immunoserological transition to PF during the follow-up period were retrospectively evaluated regarding their demographical, clinical, and laboratory characteristics. Results Among 453 patients diagnosed with PV, 13 (2.9%) patients exhibited clinical and immunoserological transition from PV to PF. The mean age of PV patients at the time of diagnosis was 39.8 ± 14.7 (19‒62) years and 7 (53.8%) of them were female. These patients showed clinical and immunoserological transition from PV to PF after a period ranging from 4 months to 13 years (mean 36.2 ± 41 months). In addition to typical clinical features of PF, all patients had positive anti-desmoglein-1 and negative anti-desmoglein-3 antibody levels after the clinical transition had occurred without any mucosal involvement. During a mean 7.8 ± 5.8 (2‒21) years of follow-up period after the transition from PV to PF, only one female patient had experienced a re-transition to PV characterized by a relapse of disease involving mucosal surfaces with positive anti-desmoglein-3 antibody levels following a 5-year period of remission period without treatment. Study limitations Single-center study with a retrospective study design. Conclusion Our series is the largest group of patients reported to show the transition from PV to PF to date with a long follow-up period. The reason behind the disappearance of anti-desmoglein-3 antibodies and the pathogenesis of this phenomenon is not yet elucidated.

Comorbidities of bullous pemphigoid: A single-center retrospective case-control study from Turkey.

Although significant associations between bullous pemphigoid (BP) and certain comorbidities, primarily subtypes of neurological disorders, have been reported in several populations, it has yet to be demonstrated whether a correlation exists between pre-existing comorbidities and serum titers of anti-BP180 and 230 immunoglobulin G (IgG) antibodies among BP patients. The aim of the current study is to investigate the demographic and clinical features of BP patients in a large series from Turkey, determine the prevalence of pre-existing neurological and systemic disorders, and assess the correlation between the existence of certain comorbidities and basal serum titers of anti-BP180 and 230 IgG autoantibodies. Thus, data from 145 BP patients diagnosed in the study's center between 1987 and 2017 were retrospectively analyzed and compared with 310 age- and sex-matched control subjects. The serum titers of anti-BP 180 and 230 IgG autoantibodies were compared between the patients with and without comorbidities and its subtypes among 55 patients with available serum basal anti-BP levels. Twenty-eight of the BP patients (19.3%) had already been diagnosed with at least one neurological disorder at the onset of BP. According to regression analysis, preexisting neurological disorders (p = 0.017), stroke (p = 0.017), and malignancies (p = 0.005) were found to be higher among the study's BP patients than the controls. The serum titers of anti-BP180 and 230 that were measured at the time of diagnosis were significantly higher in patients with neurological disorders than in patients without neurological disorders (p = 0.042; p = 0.018). Among the pre-existing comorbidities, neurological disorders, particularly stroke, and malignancies were found to be significantly connected to the occurrence of BP in the selected Turkish population. The high titers of serum anti-BP180 and 230 IgG antibodies at the time of BP diagnoses may highlight undiagnosed pre-existing neurological disorders by provoking suspicion.

Evaluation of non-endemic pemphigus foliaceus in a large series of patients: a single-center retrospective study from Turkey focuses on the relapses.

BACKGROUND: Pemphigus foliaceus is exceedingly rare around the world, except within the few regions where it occurs as an endemic variant. Various factors can trigger immune mechanisms that induce pemphigus foliaceus or worsen its course. OBJECTIVE: To determine the demographic and clinical characteristics of the patients with pemphigus foliaceus in a large series from a non-endemic country, investigate the triggering factors, and seasonal patterns. METHODS: The data of the patients diagnosed with pemphigus foliaceus in the study's center between 1989-2018 were retrospectively analyzed. RESULTS: Sixty-eight patients (mean age, 45.7 ± 14.5 years) were included in the study. The number of onsets reached its peak in spring-summer (p = 0.008). A total of 117 relapses occurred in 42 patients and were most common in spring-summer (not significant). Specific trigger factors were detected in 45 relapses. In the other 72 relapses, the peak was observed in spring-summer (p = 0.005). There were no significant differences in the demographic and clinical variables investigated between relapsed and non-relapsed patients. STUDY LIMITATIONS: Retrospective design. CONCLUSIONS: Triggering factors could not be identified in more than half of the relapses in the study's series. The subgroup of relapses (without identified causes), as well as the onsets of the disease, showed a significant seasonal variation with a peak in spring-summer; however, the seasonal variable did not justify the total group of relapses. Although the seasonal variation may be caused by a combination of factors, UV radiation should be considered a trigger factor for the peaks in spring-summer, particularly in Turkey.