Generalized pustular psoriasis with deficiency of interleukin‐36 receptor antagonist associated with sensorineural hearing impairment

Author(s):  
Susumu Ichiyama ◽  
Kazumitsu Sugiura ◽  
Toshihiko Hoashi ◽  
Naoko Kanda ◽  
Hidehisa Saeki
2015 ◽  
Vol 7 (1) ◽  
pp. 29-35 ◽  
Author(s):  
Chiharu Tominaga ◽  
Masaaki Yamamoto ◽  
Yasutomo Imai ◽  
Kiyofumi Yamanishi

A 78-year-old woman who had been suffering from psoriasis vulgaris for 31 years was admitted to hospital because of her erythroderma. A toxic eruption was suspected and she was treated with prednisolone 30 mg daily. However, it was ineffective and, suspecting psoriatic erythroderma, cyclosporine 150 mg daily was administered with tapering of the prednisolone. Two weeks after a dose reduction of cyclosporine to 100 mg/day, erythroderma with widespread generalized pustules and fever developed. Histology of a biopsy revealed inflammatory infiltrates in the skin with a spongiform pustule of Kogoj, which was consistent with generalized pustular psoriasis (GPP). Her pustules improved with additional etretinate 20 mg/day, but the erythroderma persisted and she consulted us. Three sessions of granulocyte and monocyte apheresis once weekly were effective for her condition and decreased her serum levels of IL-6 and IL-8. She had homozygous mutations of c.[28C>T] in IL36RN which cause p.[Arg10Ter]. She is the oldest reported case of GPP with a deficiency of interleukin-36 receptor antagonist (DITRA), although GPP in DITRA has been suggested to usually occur in younger cases with no pre-existing psoriasis vulgaris.


2012 ◽  
Vol 31 (7) ◽  
pp. 1267-1273 ◽  
Author(s):  
Agnieszka Pollak ◽  
Malgorzata Mueller-Malesinska ◽  
Urszula Lechowicz ◽  
Agata Skorka ◽  
Lech Korniszewski ◽  
...  

2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Yen-Hui Chan ◽  
Tien-Chen Liu ◽  
Chun-Kang Liao ◽  
Yen-Fu Cheng ◽  
Ching-Hui Tsai ◽  
...  

Abstract Betel quid is one of the most widely used psychoactive substances, and is consumed by approximately 10% of the world’s population. In addition to its carcinogenicity, betel quid has also been reported to affect many organs, including the brain, heart, lungs, gastrointestinal tract, and reproductive organs. As betel quid contains several neurotoxic ingredients, we hypothesize that it also possesses ototoxicity and may lead to sensorineural hearing impairment (SNHI). In this study, we investigated the contribution of betel quid consumption to SNHI in a large clinical cohort, and validated the pathogenetic mechanisms in ex vivo tissue explants. We enrolled a total of 2364 volunteers, and determined their audiologic results based on Z-scores converted from their original frequency-specific hearing thresholds. Using generalized linear regression, we identified a positive correlation between betel quid consumption and the Z-scores across different frequencies. Subsequently, we explored the toxicity of arecoline, the main neuroactive component of betel quid, on tissue explants from murine cochleae. Arecoline reduced cell activity in the explant cultures and induced apoptosis in the hair cells, probably through the effects of oxidative stress. These findings have expanded the potential hazards of betel quid to common neurological disorders, and provide insights into preventive strategies against SNHI caused by neurotoxic substances.


2013 ◽  
Vol 53 (9) ◽  
pp. 732-735 ◽  
Author(s):  
Kousuke Mori ◽  
Nobuyuki Ishii ◽  
Hitoshi Mochizuki ◽  
Akitoshi Taniguchi ◽  
Kazutaka Shiomi ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document