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https://www.readbyqxmd.com/read/29705999/mild-hailey-hailey-disease-cases-with-aberrant-splicing-variants-of-atp2c1-successfully-controlled-with-excimer-light
#1
M Kono, M Niizawa, T Takeichi, Y Muro, M Akiyama
A 79-year-old Japanese male (Case 1) had had repetitive erosions on the neck and trunk every summer for 13 years (Fig. 1a). A 69-year-old Japanese female (Case 2) had had recurrent erosions on the axillae, the inframammary areas and the groin every summer since her early sixties (Fig. 1d). The lesions of both patients revealed acantholysis and dyskeratosis in the epidermis (Fig. 1c, f). Neither patient had any family history of similar skin disorders. This article is protected by copyright. All rights reserved...
April 28, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/29644210/dermoscopy-of-hailey-hailey-disease
#2
Payal Chauhan, Dilip Meena, Neirita Hazarika
No abstract text is available yet for this article.
March 2018: Indian Dermatology Online Journal
https://www.readbyqxmd.com/read/29555205/store-independent-coupling-between-the-secretory-pathway-ca-2-transport-atpase-spca1-and-orai1-in-golgi-stress-and-hailey-hailey-disease
#3
Susanne Smaardijk, Jialin Chen, Sara Kerselaers, Thomas Voets, Jan Eggermont, Peter Vangheluwe
The Secretory Pathway Ca2+ ATPases SPCA1 and SPCA2 transport Ca2+ and Mn2+ into the Golgi and Secretory Pathway. SPCA2 mediates store-independent Ca2+ entry (SICE) via STIM1-independent activation of Orai1, inducing constitutive Ca2+ influx in mammary epithelial cells during lactation. Here, we show that like SPCA2, also the overexpression of the ubiquitous SPCA1 induces cytosolic Ca2+ influx, which is abolished by Orai1 knockdown and occurs independently of STIM1. This process elevates the Ca2+ concentration in the cytosol and in the non-endoplasmic reticulum (ER) stores, pointing to a functional coupling between Orai1 and SPCA1...
March 16, 2018: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29467079/narrowband-uv-b-phototherapy-in-the-treatment-of-generalized-hailey-hailey-disease
#4
M C Abaca, L Flores, V Parra
No abstract text is available yet for this article.
February 18, 2018: Actas Dermo-sifiliográficas
https://www.readbyqxmd.com/read/29344611/variable-response-to-naltrexone-in-patients-with-hailey-hailey-disease
#5
Severine Cao, Evelyn Lilly, Steven T Chen
No abstract text is available yet for this article.
March 1, 2018: JAMA Dermatology
https://www.readbyqxmd.com/read/29236657/psoriasiform-hailey-hailey-disease-presenting-as-erythematous-psoriasiform-plaques-throughout-the-body-a-case-report
#6
Jonathan Ni, Kathleen E Gilbert, Ivie F Manalo, Jashin J Wu
INTRODUCTION: Hailey-Hailey disease is an adult-onset skin condition characterized by lesions in the intertriginous regions of the body. The lesions can be pruritic, painful, and associated with physical and social impairment. CASE PRESENTATION: We present a case of psoriasiform Hailey-Hailey disease in a 60-year-old white woman who exhibited erythematous psoriasiform plaques in many areas of her body. The patient's condition was successfully treated with a twice-daily regimen of doxycycline, mometasone, and clindamycin...
2017: Permanente Journal
https://www.readbyqxmd.com/read/29139371/hailey-hailey-disease-presenting-as-lichenoid-plaques-on-the-thigh
#7
Anupama Ghosh, Anupam Das, Piyush Kumar, Swapan Sardar
A 40-year-old woman presented with multiple itchy violaceous papules and plaques on the medial aspects of both thighs. This was accompanied by a burning sensation on friction and increased itching that interfered with her daily activities, particularly in the summer. The family history was noncontributory. Cutaneous examination showed multiple flat-topped hyperkeratotic violaceous papules and plaques, appearing in linear fashion on the medial aspects of the thighs bilaterally (Figure 1). Histopathologic examination showed acanthosis, widespread suprabasal acantholysis resembling a "dilapidated brick wall," without any evidence of dyskeratosis...
2017: Skinmed
https://www.readbyqxmd.com/read/29104283/identification-of-2-novel-mutations-in-atp2c1-gene-in-hailey-hailey-disease-and-a-literature-review-of-variations-in-a-chinese-han-population
#8
Kejia Xu, Bingjun Shi, Qingchun Diao, Xue Jiang, Yujuan Xiao
BACKGROUND Hailey-Hailey disease (HHD) is a rare autosomal dominant skin condition. The ATP2C1 gene was identified as the defective gene in HHD. To date, 166 pathogenic mutations in ATP2C1 have been observed worldwide. The aim of this study was to identify variations in HHD and summarize the features of the mutations identified in China. MATERIAL AND METHODS We examined 2 familial and 2 sporadic cases of HHD. Genomic DNA polymerase chain reaction and direct sequencing of the ATP2C1 were performed from HHD patients, unaffected family members, and 200 healthy individuals...
November 6, 2017: Medical Science Monitor Basic Research
https://www.readbyqxmd.com/read/29044476/treatment-of-hailey-hailey-disease-with-botulinum-toxin
#9
Olivia A Charlton, Thomas J Stewart, Robert H Rosen
No abstract text is available yet for this article.
October 18, 2017: Australasian Journal of Dermatology
https://www.readbyqxmd.com/read/28991360/low-dose-naltrexone-a-novel-treatment-for-hailey-hailey-disease
#10
V Campbell, C McGrath, A Corry
Hailey-Hailey disease (chronic benign familial pemphigus) is a rare inherited dermatosis typically characterized by erosions at intertriginous sites preceded by minor trauma or stress. We report a case of treatment-resistant Hailey-Hailey disease having failed topical and oral steroids, prophylactic aciclovir and doxycycline, and systemic therapies including dapsone, acitretin and ciclosporin. Low-dose naltrexone 4·5 mg once daily was commenced following an incidental benefit in this patient's similarly affected sister...
October 9, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28966528/a-case-of-hailey-hailey-disease-with-a-novel-nonsense-mutation-in-the-atp2c1-gene
#11
Hazuki Yasuda, Nobuo Kanazawa, Mitsuhiro Matsuda, Takahiro Hamada, Minao Furumura, Takashi Hashimoto, Takekuni Nakama, Fukumi Furukawa
No abstract text is available yet for this article.
October 2017: Annals of Dermatology
https://www.readbyqxmd.com/read/28782140/dermoscopic-and-reflectance-confocal-microscopic-presentation-of-hailey-hailey-disease-a-case-series
#12
A Oliveira, E Arzberger, B Pimentel, V C de Sousa, P Leal-Filipe
BACKGROUND/PURPOSE: Hailey-Hailey disease is a rare inherited acantholytic skin disorder characterized by heterogeneous clinical presentation. Its differential diagnosis might be wide, including other genodermatoses, inflammatory, and infectious skin diseases. Although histopathology remains as diagnostic gold standard, noninvasive techniques such as dermoscopy and reflectance confocal microscopy may assist clinical examination. Herein, we aim to further characterize the dermoscopic and reflectance confocal microscopic presentation of Hailey-Hailey disease with histologic correlation...
February 2018: Skin Research and Technology
https://www.readbyqxmd.com/read/28768314/low-dose-naltrexone-treatment-of-familial-benign-pemphigus-hailey-hailey-disease
#13
Omer Ibrahim, Sara R Hogan, Alok Vij, Anthony P Fernandez
Importance: Familial benign pemphigus, or Hailey-Hailey disease (HHD), is a rare and debilitating genetic dermatosis characterized by chronic, recurrent vesicles, erosions, and maceration in flexural areas. Despite the reported therapeutic modalities, such as topical and systemic corticosteroids, systemic immunomodulators, topical and systemic retinoids, and laser, HHD can still be markedly difficult to control. Objective: To assess low-dose naltrexone hydrochloride in the treatment of recalcitrant HHD...
October 1, 2017: JAMA Dermatology
https://www.readbyqxmd.com/read/28768313/treatment-of-hailey-hailey-disease-with-low-dose-naltrexone
#14
Lauren N Albers, Jack L Arbiser, Ron J Feldman
Importance: Hailey-Hailey disease is a severe genetic blistering disease of intertriginous skin locations that can lead to poor quality of life and increased morbidities. Multiple therapies are available with inconsistent outcomes and potentially severe adverse effects. Objective: To determine whether low-dose naltrexone is an effective treatment for Hailey-Hailey disease. Design, Setting, and Participants: This study was a case series performed at a dermatology outpatient clinic of 3 patients with severe Hailey-Hailey disease recalcitrant to at least 4 therapies...
October 1, 2017: JAMA Dermatology
https://www.readbyqxmd.com/read/28764581/skin-diseases-of-the-vulva-inflammatory-erosive-ulcerating-and-apocrine-gland-diseases-zinc-and-vitamin-deficiency-vulvodynia-and-vestibulodynia
#15
Freja Lærke Sand, Simon Francis Thomsen
Chronic, inflammatory and ulcerating mucocutaneous diseases that can affect the vulvar area are reviewed: lichen sclerosus, lichen planus, plasma cell vulvitis, complex aphthosis, Behcet's disease, pyoderma gangrenosum, metastatic Crohn's disease, dyskeratotic skin diseases (Hailey-Hailey disease and Darier's disease), autoimmune bullous diseases (mucous membrane pemphigoid and pemphigus vulgaris) and hidradenitis suppurativa. Also, vulvodynia and vestibulodynia, zinc and vitamin B deficiency are described.
February 2018: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/28669425/-treatment-of-hailey-hailey-disease-with-botulinic-toxin-a-retrospective-study-of-8-cases
#16
L Dousset, A Pham-Ledard, M-S Doutre, M Beylot-Barry, O Cogrel
BACKGROUND: Hailey-Hailey disease (HHD) is characterised by episodes of weeping erythematous lesions, particularly in areas subject to friction or maceration. Treatment is complex. The value of botulinum toxin has been demonstrated in several studies and in individual cases. AIM: To report clinical and progressive data for 8 patients treated for HHD with injections of botulinum toxin A (BTX-A), following the failure of several other therapeutic approaches. PATIENTS AND METHODS: Eight patients (three males and five females), of median age 52...
October 2017: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/28653466/hailey-hailey-disease-due-to-atp2c1-splice-site-mutation-successfully-treated-with-minocycline-hydrochloride
#17
M Kono, M Niizawa, T Takeichi, Y Muro, M Akiyama
Hailey-Hailey disease (HHD) is an autosomal dominant disease characterized by acantholysis and caused by ATP2C1.(1) Here, we report the first case of HHD successfully treated with minocycline alone in which the diagnosis was confirmed by gene analysis. This article is protected by copyright. All rights reserved.
June 27, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28551824/the-role-of-the-atp2c1-gene-in-hailey-hailey-disease
#18
REVIEW
Hao Deng, Heng Xiao
Hailey-Hailey disease (HHD) is a rare autosomal dominant acantholytic dermatosis, characterized by a chronic course of repeated and exacerbated skin lesions in friction regions. The pathogenic gene of HHD was reported to be the ATPase calcium-transporting type 2C member 1 gene (ATP2C1) located on chromosome 3q21-q24. Its function is to maintain normal intracellular concentrations of Ca2+ /Mn2+ by transporting Ca2+ /Mn2+ into the Golgi apparatus. ATP2C1 gene mutations are reportedly responsible for abnormal cytosolic Ca2+ /Mn2+ levels and the clinical manifestations of HHD...
October 2017: Cellular and Molecular Life Sciences: CMLS
https://www.readbyqxmd.com/read/28541878/dermatosis-papular-acantol%C3%A3-tica-de-la-vulva-con-buena-respuesta-a-tacr%C3%A3-limus-t%C3%A3-pico
#19
Miguel Angel Flores-Terry, Pamela Zamberk Majlis, Monserrat Franco-Muñoz, Elena Vera-Iglesias, Mónica García-Arpa, Francisco Martín-Dávila
Papular acantholytic dermatosis of the vulva is a rare, chronic disorder and is an entity that remains to be fully understood. It shares clinical and histopathological overlap with Darier disease and Hailey-Hailey disease. We describe a 30-year-old woman with papular acantholytic dermatosis of the vulva. The lesions consisted of whitish papules and erosions on the labia majora. Histologically, there was hyperkeratosis and focal parakeratosis with acantholytic and dyskeratotic cells. She did not respond completely to topical steroids but clinical improvement occurred after the use of topical tacrolimus...
April 15, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/28507500/the-coexistence-of-darier-s-disease-and-hailey-hailey-disease-symptoms
#20
Katarzyna A Tomaszewska, Zofia Gerlicz-Kowalczuk, Magdalena Kręgiel, Marcin Noweta, Katarzyna Płużańka-Srebrzyńska, Anna Żuchowska, Andrzej Kaszuba
No abstract text is available yet for this article.
April 2017: Postȩpy Dermatologii i Alergologii
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