Generalized pustular psoriasis (GPP) is a rare inflammatory skin disease that can be life-threatening. Recently, it has been reported that familial GPP is caused by homozygous or compound ...heterozygous mutations of IL36RN. However, the majority of GPP cases are sporadic and it is controversial whether IL36RN mutations are a causative/predisposing factor for sporadic GPP. We searched for IL36RN mutations in two groups of GPP patients in the Japanese population in this study: GPP without psoriasis vulgaris (PV), and GPP with PV. Eleven cases of GPP without PV (GPP alone) and 20 cases of GPP accompanied by PV (GPP with PV) were analyzed. Surprisingly, 9 out of 11 cases of GPP alone had homozygous or compound heterozygous mutations in IL36RN. In contrast, only 2 of 20 cases of GPP with PV had compound heterozygous mutations in IL36RN. The two cases of GPP with PV who had compound heterozygous mutations in IL36RN are siblings, and both cases had PV-susceptible HLA-A*0206. We determined that GPP alone is a distinct subtype of GPP and is etiologically distinguished from GPP with PV, and that the majority of GPP alone is caused by deficiency of the interleukin-36 receptor antagonist due to IL36RN mutations.
IgG4‐related disease (IgG4‐RD) is a recently designated disease entity and its full picture has not yet been elucidated. Here, we report an unusual case of a patient with gastric wall thickening ...secondary to IgG4‐RD. A 68‐year‐old male visited our hospital with itchy skin lesions and an episode of organizing pneumonia. On the suspicion of malignancy‐associated skin lesions, computed tomography (CT) was performed. The CT revealed prominent thickening of the gastric wall. Due to the possibility of malignancy, the patient underwent distal gastrectomy. Histopathological examination showed fibrosis of the submucosa and prominent thickening of the muscularis propria. Most of infiltrating cells were IgG4‐positive plasma cells. Post‐operative blood test revealed significantly high serum levels of total IgG and IgG4. Based on these histological features, the patient was given a definitive diagnosis of IgG4‐RD. Further accumulation of cases like the present case that develop IgG4‐RD with rare manifestations would lead to the elucidation of pathogenesis.
The efficacy of biologics in psoriasis treatment is clinically proven; however, biologics are expensive. In this study, we assessed the real‐world cost‐effectiveness of biologics for psoriasis ...treatment by evaluating the relationship between biologic drug survival (DS) and total medical‐treatment costs from a pharmacoeconomic viewpoint. Furthermore, the effects of patient factors on cost‐effectiveness were investigated. We retrospectively reviewed the medical records of 135 cases who received either a tumor necrosis factor‐alpha (TNF‐α) monoclonal antibody (TNF‐mab), interleukin (IL)‐17 mab, or IL23p19‐mab for psoriasis from January 2010 to June 2020 at Yamaguchi University Hospital. We compared the monthly medical‐treatment costs according to biologic classification and found that costs of medical services, tests, and external preparations required for the treatment process were significantly higher in the TNF‐mab group than in the other groups, and the total medical costs associated with TNF‐mab treatment were significantly higher than those of IL17‐mab treatment. The total monthly cost of medical care was lower in the long‐term DS group than in the short‐term group. The number of prescriptions for external preparations, comprising Vitamin D3 and corticosteroid, was significantly higher in the long‐term DS group than in the short‐term group; in the TNF‐mab group, the proportion of patients without smoking habits was significantly higher in the long‐term group as well. Our study indicated that when costly biologics are used for psoriasis treatment, the maintenance of long‐term DS and appropriate patient guidance might improve the quality of medical care, thus allowing cost‐effective medical care.
Although rare, tuberculosis has been reported with biologic treatment against psoriasis in Japan, a tuberculosis medium‐burden country. Mycobacterial infection often develops after a long incubation ...period and might not have been adequately identified in clinical trials or post‐marketing surveillance. To determine the real‐world incidence of tuberculosis in psoriatic patients treated with biologics, we conducted a retrospective, multicenter, observational study in 18 facilities in Western Japan. Psoriatic patients who visited a participating facility between 2010 and March 2017 and received biologic reagents were enrolled. Information on sex, age at first biologic treatment, results of interferon‐γ release assay (IGRA) for Mycobacterium tuberculosis, treatment history with isoniazid, and onset of active and/or latent tuberculosis was collected. A total of 1117 patients (830 men and 287 women) were enrolled. The mean duration of biologic treatment was 3.54 years. Sixty‐five patients (5.8%) showed positive IGRA results at screening. Active tuberculosis developed in two patients after the administration of tumor necrosis factor inhibitors (both involved miliary tuberculosis). Latent tuberculosis was observed in two patients treated with anti‐interleukin‐12/23p40 antibody. The incidence rate of tuberculosis, including latent tuberculosis, in this survey was 0.36%. Although the incidence rate of tuberculosis was low considering the observation period of biologic treatment, active tuberculosis was found in both the screening‐negative group and a screening‐positive subject after isoniazid prophylaxis (both miliary tuberculosis), concluding that negative screening or isoniazid treatment does not always assure that an individual has no tuberculosis. Hence, dermatologists still need to pay careful attention to tuberculosis at every patient visit.
Psoriatic arthritis (PsA) is an inflammatory arthritis with as yet unclear pathophysiology. This retrospective, multicenter, cross‐sectional study was conducted in 19 facilities in western Japan and ...aimed to identify patients’ characteristics and factors that affect the results of treatment with biologic agents. Of 2116 patients with psoriasis, 285 (13.5%) had PsA. Skin manifestations preceded joint manifestations in 69.8%, the onset was simultaneous in 17.2%, whereas PsA preceded skin manifestations in 2.5%. Peripheral arthritis was most common, occurring in 73.7%, compared with axial disease in 21.8%, enthesitis in 23.5% and dactylitis in 35.4%. Patients with severe skin manifestations were significantly younger at onset (P = 0.02) and more frequently had axial disease (P < 0.01). Biologic agents were used in 206 patients (72.3%), anti‐tumor necrosis factor (TNF)‐α antibodies being prescribed first to 157 of them. Anti‐TNF‐α antibodies were continued by 105 participants and discontinued by 47, the remaining five patients being lost to follow up. Patients who discontinued anti‐TNF‐α antibodies were significantly older than those who continued (55 vs 51 years, P = 0.04) and significantly older at onset of joint manifestations (50 vs 44 years, P = 0.01). Multivariate analysis revealed that patients over 50 years significantly more frequently terminated anti‐TNF‐α antibodies (P < 0.01). In conclusion, patients with PsA and severe skin manifestations have earlier onset and axial disease, which seriously impacts on quality of life. Anti‐TNF‐α antibodies were generally effective enough to continue but less so in patients aged over 50 years. Further detailed research is needed.
On physical examination, multiple, red-brown, annular keratotic lesions measuring up to 1 cm in diameter were symmetrically distributed on sun-exposed areas of the extremities Figure 1a. Some scaly ...plaques were observed on the face but the trunk was not involved. 1 Since then, various treatment options for this condition including topical diclofenac, vitamin D 3 analogs, 5-fluorouracil, retinoids, 5% imiquimod, photodynamic therapy, carbon dioxide laser and oral retinoids have been reported with varying degrees of success.
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