Unique characteristics in Japanese dermatitis herpetiformis

Ohata, Chika; Ishii, Norito; Niizeki, Hironori; Shimomura, Yutaka; Furumura, Minao; Inoko, Hidetoshi; Mitsunaga, Shigeki; Saiki, Megumi; Shigeta, Mika; Fujiwara, Sakuhei; Yamakawa, Kentaro; Kobayashi, Satomi; Kamata, Masahiro; Inaba, Mayumi; Ito, Taisuke; Uhara, Hisashi; Watanabe, Rei; Ohtoshi, Shinpei; Ohashi, Takenobu; Tanaka, Toshihiro; Suzuki, Masayuki; Sitaru, Cassian; Kárpáti, Sarolta; Zone, John J.; Hashimoto, Takashi

doi:10.1111/bjd.13965

Cited by 16 publications

(17 citation statements)

References 15 publications

(35 reference statements)

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“…Although DH is a relatively common disease in Caucasian populations, it is rare in Japan. Most Japanese patients with DH show unique features, including a high frequency of fibrillar IgA deposition in the papillary dermis, rare occurrence of GSE, absence of the HLA-DQ2/DQ8 haplotype and rare associations with autoimmune disease and lymphoma, although the histological findings appear to be similar to those of granulartype DH (3,4). Furthermore, as reported previously, these patients appear to have IgA antibodies to eTG, but not tTG (5).…”

supporting

confidence: 53%

Increased Serum Levels of Th2-type Cytokines and Eotaxin in Fibrillar-type Dermatitis Herpetiformis

Makino

Yoshihisa²,

Mizawa³

et al. 2017

Acta Derm Venerol

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Dermatitis herpetiformis (DH) is a pruritic papulovesicular disease that is symmetrically distributed over the shoulders, elbows, back and knees. Immunofluorescence has revealed granular IgA deposition in the papillary dermis. IgA antibodies to both epidermal transglutaminase (eTG) and tissue transglutaminase (tTG) are usually detected in patients with DH. DH is commonly thought to be a cutaneous manifestation of gluten-sensitive enteropathy (GSE) (1, 2). We refer to this condition as "granular-type DH". Although DH is a relatively common disease in Caucasian populations, it is rare in Japan. Most Japanese patients with DH show unique features, including a high frequency of fibrillar IgA deposition in the papillary dermis, rare occurrence of GSE, absence of the HLA-DQ2/DQ8 haplotype and rare associations with autoimmune disease and lymphoma, although the histological findings appear to be similar to those of granulartype DH (3, 4). Furthermore, as reported previously, these patients appear to have IgA antibodies to eTG, but not tTG (5). We refer to this condition as "fibrillar-type DH". The clinical and histological findings of typical fibrillar-type DH are shown in Fig. S1 1 . Fibrillar-type DH appears to be rare in Caucasian populations (6). While the pathogenesis of DH is unclear, previous studies have suggested that the expression of interleukin (IL)-8, granulocyte-macrophage colony-stimulating factor (GM-CSF) and Th2-type cytokines could be important in the tissue infiltration process of eosinophils and neutrophils in granular-type DH (7,8). The present study investigated the levels of serum cytokines and chemokines in order to clarify their presence and activity in the pathogenesis of fibrillar-type DH. MATERIALS AND METHODS (see Appendix S11 ) RESULTSThe study population included 5 patients with fibrillartype DH (mean age 67.4 years; age range 41-81 years) in the active stage of the disease, who had not received any treatment and in whom the development of new lesions had been observed ( Table I). The levels of IgA antibodies against eTG were markedly increased in all patients. However, no IgA antibodies against tTG were detected in any of the patients. None of the patient's intestinal tissue specimens showed villous atrophy or lymphocytic cell infiltration.The serum level of interferon (IFN)-γ, a Th1-like cytokine, was increased slightly in the patients with DH (mean 0.414 ± 0.23; range 0.21-0.80 pg/ml) in comparison with the control subjects; however, the difference was not statistically significant. IL-12, another Th1-like cytokine, was not detected in either the patients with DH or the control subjects (Fig. S2a 1 ). The patients' serum levels of Th2-like cytokines IL-4 (mean14.55 ± 5.3 pg/ml; range 6.84-21.71 pg/ml, p < 0.005), IL-5 (mean 6.09 ± 0.99 pg/ml; range 4.84-7.49 pg/ml, p < 0.001) and IL-13 (mean 1.14 ± 0.20 pg/ml; range 0.85-1.36 pg/ml, p < 0.005) were significantly increased in comparison with the normal subjects: IL-4: mean 0.04 ± 0.09 pg/ml; range 0-0.21 pg/ml, IL-5: mean 0.55 ± 0.14 p...

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supporting

confidence: 53%

Increased Serum Levels of Th2-type Cytokines and Eotaxin in Fibrillar-type Dermatitis Herpetiformis

Makino

Yoshihisa²,

Mizawa³

et al. 2017

Acta Derm Venerol

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“…DH is a rare disease with an incidence that varies between di erent geographical areas. While it is relatively more common in Northern Europe [2,8] and in the US [9], DH is very rare in almost all the other countries, being exceptional in the Far East, although some case series of patients with DH were previously reported in the literature [10], especially from Japan [11,12]. In Italy, the exact incidence of DH is not known, although it is considered a relatively high incidence country [13].…”

Section: Discussionmentioning

confidence: 99%

Female Patients with Dermatitis Herpetiformis Show a Reduced Diagnostic Delay and Have Higher Sensitivity Rates at Autoantibody Testing for Celiac Disease

Antiga

Bonciolini

Cazzaniga

et al. 2019

BioMed Research International

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Objective. Our objective was to characterize the demographic information, clinical features, and laboratory data of patients with dermatitis herpetiformis (DH). Methods. In this multicentre cross-sectional study, consecutive patients with a new diagnosis of DH that referred to nine di erent Italian centers between 2011 and 2016 were characterized assessing demographic, clinical and laboratory ndings, and evaluating gender and age di erences across selected variables. Results. A total of 151 patients were included. Among them, 81 (53.6%) were males and 70 (46.4%) were females, with a male to female ratio of 1.2 : 1. e median age at the time of diagnosis was 41 years (range 0-85). Males had a signi cant longer diagnostic delay if compared to females (9 vs. 3 months, respectively; = 0.01). Direct immuno uorescence was positive in 94.7% of the patients, while duodenal biopsy showed partial to total villous atrophy in 70.1% of patients. All the females resulted positive to at least one of the antibodies tested, while a total of 12 male patients (10.5%) tested negative to celiac-speci c antibodies. Female patients had a high rate (14.1%) of autoimmune thyroiditis. Conclusions. Our study con rmed some of the most relevant data regarding DH that have been previously reported in the literature. In addition, we found a reduced diagnostic delay in females with respect to males, possibly related to the higher sensitivity of serologic testing in females with DH compared to males. Finally, we demonstrated that intestinal involvement could be severe in patients with DH and that females should be tested for thyroiditis.

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“…As we have recently reported, patients with DH are very rare in Japan, presumably due to different frequencies of pathogenic human leucocyte antigens. 59,89 Indeed, both the clinical and final diagnoses in this study included very few patients with DH, with around 16 cases.…”

Section: Discussionmentioning

confidence: 99%

“…All sera were kept at −80 °C for long durations and at 4 °C with 0·1% sodium azide as a preservative during experiments. Studies of the considerably large number of patients used in this study have previously been reported . All previously reported studies for smaller numbers of patients used in this study are listed in Appendix S2 (see Supporting Information).…”

Section: Methodsmentioning

confidence: 99%

Summary of results of serological tests and diagnoses for 4774 cases of various autoimmune bullous diseases consulted to Kurume University

et al. 2016

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Unique characteristics in Japanese dermatitis herpetiformis

Cited by 16 publications

References 15 publications

Increased Serum Levels of Th2-type Cytokines and Eotaxin in Fibrillar-type Dermatitis Herpetiformis

Increased Serum Levels of Th2-type Cytokines and Eotaxin in Fibrillar-type Dermatitis Herpetiformis

Female Patients with Dermatitis Herpetiformis Show a Reduced Diagnostic Delay and Have Higher Sensitivity Rates at Autoantibody Testing for Celiac Disease

Summary of results of serological tests and diagnoses for 4774 cases of various autoimmune bullous diseases consulted to Kurume University

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