Pustulotic arthro-osteitis (PAO) is sometimes associated with palmoplantar pustulosis; however, it is still debated whether PAO is a synonym of SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome or a different disease. In order to investigate how Japanese dermatologists currently understand the pathogenesis of these two conditions, a questionnaire survey was sent to the professors and chairmen of 80 universities. The response rate was 93%. The results showed that the percentage of respondents who distinguish between the terms PAO and SAPHO syndrome (66%) was 4 times higher than that of those who do not (16%). Nearly half of the respondents stated that PAO is included in SAPHO syndrome, but one-third of the respondents considered PAO to be different from SAPHO syndrome. More than 70% of the respondents answered that they do not agree that PAO has occasionally been reported as SAPHO syndrome by Japanese dermatologists. Regarding therapies for PAO, 15% of the respondents (n=11) used biologics, including adalimumab (n=9), infliximab (n=4), and ustekinumab (n=1), all of which resulted in favorable effects. Cases having all the features of synovitis, acne, pustulosis, hyperostosis, and osteitis were observed in 4 institutes. Because PAO associated with palmoplantar pustulosis is frequently observed in our country, Japanese dermatologists should take the initiative to clarify the similarities and differences between PAO and SAPHO syndrome.
The incidence of bullous pemphigoid (BP) has been reported in several countries, but there are no available data in Japan. To estimate the incidence of BP in Japan and compare it with those reported from other countries, we retrospectively collected BP cases in the southern part of Miyagi prefecture, which has a total population of 1.354 million inhabitants. These cases were diagnosed between January 2008 and December 2012 at all dermatology clinics and dermatology branches of 2 general hospital located in that area. A total of 75 BP cases (28 male and 47 female) were identified by at least clinical findings and an increased serum level of anti-BP180 antibody. The overall estimated incidence was 42.1 cases per million persons per year in males and 68.2 cases in females. In the population aged 80 years or above, the BP incidence was 642.4 cases per million per year in males and 606.3 cases in females. When we subdivided the cases whose diagnoses were confirmed histologically, the number of cases was 11 for males and 18 for females. Therefore, the overall BP incidence was 16.5 cases per million persons per year in males and 26.1 cases in females. In the population aged 80 years or above, BP incidence was 224.9 cases per million per year for males and 245.8 cases for females. These data suggested that the incidence of BP in Japan is higher than those reported in other countries.
Streptococcal toxic shock syndrome (STSS) has a high mortality rate, and group A streptococcus is the usual causative bacterial species. Recently, cases of STSS caused by non-group A β-hemolytic streptococcus have increased. According to the Japan-Infections Disease Law, reporting all cases of STSS caused by β-hemolytic streptococcus has been mandatory since 2006.
In contrast, necrotizing fasciitis, which is a frequent complication with STSS, but not the same disease, is not required to be reported. Therefore, case reports of necrotizing fasciitis are sporadic. In this report, we collected case reports of necrotizing fasciitis between 2011 and 2015 in Japan and based on these reports, we reconsidered the existing definition of necrotizing fasciitis, clarified the characteristics of non-group A streptococcal necrotizing fasciitis, and determined the appropriate operative interventions for the treatment of the condition.