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https://www.readbyqxmd.com/read/28395432/-clinicopathological-features-of-acantholytic-mammary-paget-s-disease-a-report-of-28-cases
#1
Y P Zeng, C Chen, K Fang, Q N Jia, D L Ma
Objective: To investigate the clinicopathological features of acantholytic mammary Paget's disease (AMPD). Methods: From January, 2010 to October, 2016, a total of 28 patients were diagnosed as AMPD in the Department of Dermatology, Peking Union Medical College Hospital. The clinical and histopathological data of these patients were analyzed retrospectively. Results: The patients were all female. The mean age of onset was (51±15)years (range, 24 to 78 years). The median course of disease was 10.5 months (range, 3 months to 2 years)...
April 11, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28326204/an-unusual-location-of-subungual-warty-dyskeratoma-a-case-report-and-review-of-the-literature
#2
Elena Vargas-Laguna, Adrián Imbernón-Moya, Antonio Aguilar-Martínez, Fernando Burgos
Warty dyskeratoma is an uncommon entity characterized by a solitary keratotic papule or nodule usually located in the head and neck of young adults. The histopathology shows a pattern of acantholytic dyskeratosis. We report a 32-year-old man who presented pain, serous exudation, a distal onycholysis with subungual hyperkeratosis, and roundish erythronychia in the nail plate of his left first toe 2 years ago. A histopathologic diagnosis of subungual warty dyskeratoma was made. When dealing with focal acantholytic dyskeratosis several differential diagnoses should be considered including Darier's disease, transient focal acantholytic dyskeratosis or Grover disease, and Hailey-Hailey disease...
2017: Case Reports in Dermatological Medicine
https://www.readbyqxmd.com/read/28301978/is-photodynamic-therapy-a-relevant-therapeutic-option-in-refractory-benign-familial-pemphigus-hailey-hailey-disease-a-series-of-eight-patients
#3
Maha Alsahli, Anca Debu, Celine Girard, Didier Bessis, Aurélie Du Thanh, Bernard Guillot, Olivier Dereure
INTRODUCTION/BACKGROUND: Treatment of benign familial pemphigus or Hailey-Hailey disease (HHD), a rare inherited condition associated with a significant impairment of quality of life, is often challenging and disappointing with frequent relapses and infectious complications. Topical photodynamic therapy (PDT) may offer new perspectives in this difficult setting. MATERIAL AND METHODS: Eight patients with long-lasting HHD lesions refractory to multiple treatments were treated on at least one involved site with PDT using methyl-amino levulinate with a standardized protocol of three sessions of irradiation separated by 3-week intervals...
April 2, 2017: Journal of Dermatological Treatment
https://www.readbyqxmd.com/read/28300154/corrigendum-the-loss-of-atp2c1-impairs-the-dna-damage-response-and-induces-altered-skin-homeostasis-consequences-for-epidermal-biology-in-hailey-hailey-disease
#4
Samantha Cialfi, Loredana Le Pera, Carlo De Blasio, Germano Mariano, Rocco Palermo, Azzurra Zonfrilli, Daniela Uccelletti, Claudio Palleschi, Gianfranco Biolcati, Luca Barbieri, Isabella Screpanti, Claudio Talora
No abstract text is available yet for this article.
March 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28264934/structure-activity-relationship-of-thapsigargin-inhibition-on-the-purified-golgi-secretory-pathway-ca2-mn2-transport-atpase-spca1a
#5
Jialin Chen, Joren De Raeymaecker, Jannik Brøndsted Hovgaard, Susanne Smaardijk, Ilse Vandecaetsbeek, Frank Wuytack, Jesper Vuust Møller, Jan Eggermont, Marc De Maeyer, Søren Brøgger Christensen, Peter Vangheluwe
The Golgi/secretory pathway Ca2+/Mn2+ transport ATPase (SPCA1a) is implicated in breast cancer and Hailey-Hailey disease. Here, we purified recombinant human SPCA1a from Saccharomyces cerevisiae and measured Ca2+ dependent ATPase activity following reconstitution in proteoliposomes. The purified SPCA1a displays a higher apparent Ca2+ affinity and lower maximal turnover rate than the purified sarco(endo)plasmic reticulum Ca2+ ATPase (SERCA1a). The lipids cholesteryl hemisuccinate, linole-/oleamide and phosphatidyl ethanolamine inhibit, whereas phosphatidic acid and sphingomyelin enhance SPCA1a activity...
March 6, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28260304/-clinicopathological-study-of-56-cases-of-extramammary-paget-s-disease-with-or-without-acantholysis
#6
Y P Zeng, C Chen, K Fang, Q N Jia, D L Ma
Objective: To identify the clinicopathological features of extramammary Paget's disease(EMPD) and investigate the clinical and histopathological significance of acantholysis in EMPD. Methods: From June, 2010 to October, 2016, a total of 56 patients were diagnosed as EMPD in the Department of Dermatology, Peking Union Medical College Hospital. Clinical and histopathological data were retrieved from these patients' medical records and analyzed respectively. The cases were divided into two subgroups according to the histopathological pattern (with or without acantholysis): the acantholytic EMPD (AEMPD) group and the non-acantholytic EMPD (N-AEMPD) group...
February 28, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28256765/mimickers-of-classic-acantholytic-diseases
#7
REVIEW
Jonathan Ho, Jag Bhawan
Acantholysis is a commonly encountered histological pattern which typically generates a differential of the pemphigus variants, Hailey-Hailey, Darier's and Grover's diseases. In addition to these diseases, the dermatologist and dermatopathologist must be aware of entities that mimic classic acantholytic dermatoses and of rare disease variants, which are characterized by acantholysis.
March 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28223763/the-effect-of-cryotherapy-in-hailey-hailey-disease
#8
Jee Hee Son, Yong Se Cho, Yun Sun Byun, Bo Young Chung, Chun Wook Park, Hye One Kim
No abstract text is available yet for this article.
February 2017: Annals of Dermatology
https://www.readbyqxmd.com/read/28108048/bullous-pseudobullous-pustular-dermatoses
#9
Mark R Wick
Several dermatoses are typified by the formation of spaces (blisters; bullae) within or beneath the epidermis. These may be acellular or filled with particular species of inflammatory cells. Etiological categories include infectious, immune-mediated, genetic, drug-related, and idiopathic lesions. Examples of such disorders include impetigo, Herpes virus infections, pemphigus, bullous pemphigoid and pemphigoid gestationis, epidermolysis bullosa acquisita, IgA-related dermatoses, inherited epidermolysis bullosa variants, Hailey-Hailey disease, and porphyria cutanea tarda...
December 14, 2016: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28087023/dermoscopic-presentation-of-hailey-hailey-disease
#10
Awatef Kelati, Giuseppe Argenziano, Fatima Zahra Mernissi
No abstract text is available yet for this article.
February 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28063031/pharmacokinetics-and-pharmacodynamics-of-afamelanotide-and-its-clinical-use-in-treating-dermatologic-disorders
#11
REVIEW
Elisabeth I Minder, Jasmin Barman-Aksoezen, Xiaoye Schneider-Yin
Afamelanotide, the first α-melanocyte-stimulating hormone (MSH) analogue, synthesized in 1980, was broadly investigated in all aspects of pigmentation because its activity and stability were higher than the natural hormone. Afamelanotide binds to the melanocortin-1 receptor (MC1R), and MC1R signaling increases melanin synthesis, induces antioxidant activities, enhances DNA repair processes and modulates inflammation. The loss-of-function variants of the MC1R present in fair-skinned Caucasians are less effectively activated by the natural hormone...
January 6, 2017: Clinical Pharmacokinetics
https://www.readbyqxmd.com/read/28052370/acantholytic-dermatosis-of-the-vagina-the-diagnostic-challenge-of-acantholytic-disease-in-the-genital-region
#12
J Kentley, R Cerio, M Khorshid, K Gibbon
We report the case of a 24-year-old woman with an 8-month history of deep pelvic pain and postcoital bleeding. Examination revealed desquamation of the vaginal epithelium with tender fissured plaques in the vagina, initially thought to be vaginal intraepithelial neoplasia. Histology showed squamous mucosa with suprabasal acantholysis and hyperkeratosis, and no evidence of viral infection, dysplasia or malignancy. These findings were consistent with acantholytic dermatosis (AD), a rare lesion that resembles Hailey-Hailey and Darier disease histopathologically, but can be distinguished on a clinical basis...
January 4, 2017: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/28035777/mendelian-disorders-of-cornification-caused-by-defects-in-intracellular-calcium-pumps-mutation-update-and-database-for-variants-in-atp2a2-and-atp2c1-associated-with-darier-disease-and-hailey-hailey-disease
#13
Ruud G L Nellen, Peter M Steijlen, Maurice A M van Steensel, Maaike Vreeburg, Jorge Frank, Michel van Geel
The two disorders of cornification associated with mutations in genes coding for intracellular calcium pumps are Darier disease (DD) and Hailey-Hailey disease (HHD). DD is caused by mutations in the ATP2A2 gene, whereas the ATP2C1 gene is associated with HHD. Both are inherited as autosomal-dominant traits. DD is mainly defined by warty papules in seborrheic and flexural areas, whereas the major symptoms of HHD are vesicles and erosions in flexural skin. Both phenotypes are highly variable. In 12%-40% of DD patients and 12%-55% of HHD patients, no mutations in ATP2A2 or ATP2C1 are found...
April 2017: Human Mutation
https://www.readbyqxmd.com/read/27745906/interventional-treatments-for-hailey-hailey-disease
#14
REVIEW
Benjamin Farahnik, Collin M Blattner, Michael B Mortazie, Benjamin M Perry, William Lear, Dirk M Elston
Hailey-Hailey disease or familial benign chronic pemphigus is a rare blistering dermatosis that is characterized by recurrent erythematous plaques with a predilection for the skin folds. For extensive Hailey-Hailey disease that is recalcitrant to conventional therapy, laser ablation, photodynamic therapy, electron beam radiotherapy, botulinum toxin type A, dermabrasion, glycopyrrolate, and afamelanotide have been reported as useful treatments, but comparative trials are lacking. This review discusses the various treatment modalities for Hailey-Hailey disease and a summary of the evidence for the most recommended treatments...
March 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/27695354/management-of-familial-benign-chronic-pemphigus
#15
Harleen Arora, Fleta N Bray, Jessica Cervantes, Leyre A Falto Aizpurua
Benign familial chronic pemphigus or Hailey-Hailey disease is caused by an autosomal dominant mutation in the ATP2C1 gene leading to suprabasilar acantholysis. The disease most commonly affects intertriginous areas symmetrically. The chronic nature of the disease and multiple recurrences make the disease bothersome for patients and a treatment challenge for physicians. Treatments include topical and/or systemic agents and surgery including laser. This review summarizes the available treatment options.
2016: Clinical, Cosmetic and Investigational Dermatology
https://www.readbyqxmd.com/read/27617466/familial-benign-pemphigus-atypical-localization
#16
Maria Veronica Reyes, Sabina Halac, Claudio Mainardi, Maria Kurpis, Alejandro Ruiz Lascano
We present an atypical case of familial benign pemphigus (Hailey-Hailey disease), which presented as crusted, annular plaques limited to the back without intertriginous involvement. We could not find in the literature another patient with plaques located solely on the back without a prior history of classical disease.
April 18, 2016: Dermatology Online Journal
https://www.readbyqxmd.com/read/27528123/the-loss-of-atp2c1-impairs-the-dna-damage-response-and-induces-altered-skin-homeostasis-consequences-for-epidermal-biology-in-hailey-hailey-disease
#17
Samantha Cialfi, Loredana Le Pera, Carlo De Blasio, Germano Mariano, Rocco Palermo, Azzurra Zonfrilli, Daniela Uccelletti, Claudio Palleschi, Gianfranco Biolcati, Luca Barbieri, Isabella Screpanti, Claudio Talora
Mutation of the Golgi Ca(2+)-ATPase ATP2C1 is associated with deregulated calcium homeostasis and altered skin function. ATP2C1 mutations have been identified as having a causative role in Hailey-Hailey disease, an autosomal-dominant skin disorder. Here, we identified ATP2C1 as a crucial regulator of epidermal homeostasis through the regulation of oxidative stress. Upon ATP2C1 inactivation, oxidative stress and Notch1 activation were increased in cultured human keratinocytes. Using RNA-seq experiments, we found that the DNA damage response (DDR) was consistently down-regulated in keratinocytes derived from the lesions of patients with Hailey-Hailey disease...
2016: Scientific Reports
https://www.readbyqxmd.com/read/27523793/glutathione-s-transferase-%C3%AF-subunit-as-a-phenotypic-suppressor-of-pmr1%C3%AE-strain-the-kluyveromyces-lactis-model-for-hailey-hailey-disease
#18
G Ficociello, E Zanni, S Cialfi, C Aurizi, G Biolcati, C Palleschi, C Talora, D Uccelletti
BACKGROUND: Hailey-Hailey disease (HHD), also known as familial benign chronic pemphigus, is a rare, chronic and recurrent blistering disorder, histologically characterized by suprabasal acantholysis. HHD has been linked to mutations in ATP2C1, the gene encoding the human adenosine triphosphate (ATP)-powered calcium channel pump. METHODS: In this work, the genetically tractable yeast Kluyveromyces lactis has been used to study the molecular basis of Hailey-Hailey disease...
November 2016: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/27510943/exacerbation-of-hailey-hailey-disease-by-topiramate
#19
LETTER
A Alkhalifah, H Montaudié, J-P Lacour, M Lantéri-Minet, T Passeron
No abstract text is available yet for this article.
April 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/27433827/koebnerization-of-hailey-hailey-disease-into-a-cutaneous-drug-eruption-of-acute-generalized-exanthematous-pustulosis-associated-with-systemic-symptoms
#20
Mariam B Totonchy, Jennifer M McNiff, Christopher G Bunick
We describe a 65-year-old Caucasian female with well-controlled Hailey-Hailey disease (HHD) who developed acute generalized exanthematous pustulosis (AGEP) with severe systemic symptoms. Despite sparing of the patient's intertriginous skin, histopathologic evidence of HHD was observed in all biopsies, suggestive of a unique koebernization phenomenon of HHD to areas of cutaneous drug eruption. While internal organ involvement is less commonly reported in AGEP, there are an increasing number of patients with signs and symptoms suggestive of an AGEP/drug reaction with eosinophilia and systemic symptoms (DRESS) spectrum of cutaneous drug disorders...
November 2016: Journal of Cutaneous Pathology
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