Pemphigus Foliaceus and Pemphigus Erythematosus are the Most Common Subtypes of Pemphigus in Northern Finland

Abstract

Pemphigus comprises a group of autoimmune skin diseases characterized by blistering and erosions of the skin and mucous membranes. Pemphigus is rare in Northern and Western Europe, but its incidence is higher around the Mediterranean Sea. The most common type worldwide is pemphigus vulgaris. To the best of our knowledge, there are no recent reports of the incidence of pemphigus in Nordic populations. The latest study into the incidence of pemphigus in Finland is from the 1970s. However, the incidence may have been affected, for example, by immigration. This is a retrospective database study of all patients with pemphigus diagnosed in the Oulu University Hospital between 1985 and 2017. The current study included 46 patients, and interestingly, the most prevalent subtype was superficial pemphigus foliaceus or erythematosus (65%). This study also reports comorbidities of study patients and treatments used for pemphigus in the Oulu University Hospital. Pemphigus is an autoimmune skin disease characterized by blistering and erosions of the skin and mucous membranes. Pemphigus is rare in Northern and Western Europe, but its incidence is higher around the Mediterranean Sea. The most common type worldwide is pemphigus vulgaris. The aim of this study was to investigate the incidence of pemphigus subtypes in Northern Finland between 1985 and 2017. A total of 46 patients diagnosed with pemphigus at the Department of Dermatology of Oulu University Hospital were found; the female/male ratio was 1.7. In contrast to many other countries, it was found that, in Northern Finland, the superficial pemphigus subtypes were the most common: erythematosus or foliaceus (65%) followed by pemphigus vulgaris (26%). Over the past 4 decades the annual incidence of pemphigus in Finland has increased from 0.76 to 2.8 cases per million persons.

Pemphigus erythematosus: A case series from a tertiary academic center and literature review

“…Of the remaining papers and their references, 58 documented at least one case of PE, corresponding to 226 total patients. Eight of the papers reported case‐series for a total 139 patients without providing the individual patient data 7‐14 . Those papers are briefly mentioned but were not included in statistical analyses of the demographic, clinical, and serological data reported below.…”

“…Globally, the most common form of pemphigus reported is pemphigus vulgaris (PV), with PE only consisting of approximately 7.7% to 8.7% of total pemphigus cases 7,14 . However, Försti et al noted that in Northern Finland between 1985 and 2017, PE was the most common form of pemphigus overall 13 . PE has also been reported in a significantly higher proportion of the cases in the age group of 2.5 to 20 years old when looking at all cases of pemphigus 5,7,49 .…”

Deep pemphigus (pemphigus vulgaris, pemphigus vegetans and paraneoplastic pemphigus) in dogs, cats and horses: a comprehensive review

“…Another paper specifies that PV consists of almost 90% of 180 patients with pemphigus seen at a tertiary referral dermatology center in Israel between 2000 and 2015 [ 35 ]. In contrast, a recent paper highlights that pemphigus foliaceus/erythematosus is more common than PV in northern Finland [ 36 ]. The reason for this disparity is unknown, but this could be due to a small study population or to regional genetic differences.…”

Senear-Usher syndrome in a 5-year-old girl

“…Kumar et al ., in a 2008 article, reported a high prevalence (4.4 cases per million population) [ 6 ]. However, in northern Finland, pemphigus foliaceus and pemphigus erythematosus are the most common pemphigus subtypes [ 7 ].…”