The Experts below are selected from a list of 1539 Experts worldwide ranked by ideXlab platform
S.m. Burge - One of the best experts on this subject based on the ideXlab platform.
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Calcium pumps and keratinocytes: lessons from Darier's Disease and Hailey-Hailey Disease.
The British journal of dermatology, 2004Co-Authors: Jittima Dhitavat, R J Fairclough, Alain Hovnanian, S.m. BurgeAbstract:Darier's Disease and Hailey-Hailey Disease are autosomal dominantly inherited skin disorders in which desmosomal adhesion between keratinocytes is abnormal. ATP2A2 and ATP2C1 have been identified as the causative genes for Darier's Disease and Hailey-Hailey Disease, respectively. ATP2A2 encodes the sarco(endo)plasmic reticulum Ca(2+)-ATPase isoform 2 (SERCA2) pump, while ATP2C1 encodes a secretory pathway Ca(2+)/Mn(2+)-ATPase (SPCA1) found in the Golgi apparatus. We review recent work into the function of these pumps in human keratinocytes and discuss how mutations in these genes might cause these Diseases by altering the formation or stability of desmosomes.
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handicap in darier s Disease and Hailey Hailey Disease
British Journal of Dermatology, 1996Co-Authors: A. Harris, S.m. Burge, P.j. Dykes, Andrew Yule FinlayAbstract:One hundred and thirty-seven patients with Darier's Disease and 66 patients with Hailey-Hailey Disease completed the Dermatology Life Quality Index (DLQI). The average DLQI score for Darier's Disease was 5.89 (19.6% of the maximum score), median = 4, quartiles: 1.9. The average DLQI score for Hailey-Hailey Disease was 6.06 (20.2% of the maximum score), median = 5, quartiles: 2.9. The DLQI score did not correlate with the physician's assessment of clinical severity in both Diseases, thus emphasizing the importance of remembering the potential for handicap even in patients with Disease that seems to be mild. The domains of the questionnaire with the highest mean scores in both Diseases were for questions relating to symptoms and feelings. Domains relating to personal relationships and work obtained low mean scores even in severe Disease. It is encouraging that most patients with Darier's Disease and Hailey-Hailey Disease maintain good relationships at work and home and seem to be able to adapt to their skin Disease.
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Handicap in Darier's Disease and Hailey‐Hailey Disease
The British journal of dermatology, 1996Co-Authors: A. Harris, S.m. Burge, P.j. Dykes, Andrew Yule FinlayAbstract:One hundred and thirty-seven patients with Darier's Disease and 66 patients with Hailey-Hailey Disease completed the Dermatology Life Quality Index (DLQI). The average DLQI score for Darier's Disease was 5.89 (19.6% of the maximum score), median = 4, quartiles: 1.9. The average DLQI score for Hailey-Hailey Disease was 6.06 (20.2% of the maximum score), median = 5, quartiles: 2.9. The DLQI score did not correlate with the physician's assessment of clinical severity in both Diseases, thus emphasizing the importance of remembering the potential for handicap even in patients with Disease that seems to be mild. The domains of the questionnaire with the highest mean scores in both Diseases were for questions relating to symptoms and feelings. Domains relating to personal relationships and work obtained low mean scores even in severe Disease. It is encouraging that most patients with Darier's Disease and Hailey-Hailey Disease maintain good relationships at work and home and seem to be able to adapt to their skin Disease.
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Hailey Hailey Disease the clinical features response to treatment and prognosis
British Journal of Dermatology, 1992Co-Authors: S.m. BurgeAbstract:Summary Fifty-eight individuals with Hailey–Hailey Disease were studied to delineate the clinical features, response to treatment and prognosis. The Disease generally presented between the second and fourth decades, but the morphology of lesions was varied and a delay in diagnosis was common. Nail changes have not been documented in previous studies of Hailey–Hailey Disease, but asymptomatic longitudinal white bands were present in the fingernails in 71% of 38 patients examined and are a helpful physical sign. The Disease is predominantly flexural. Friction and heat or sweating exacerbate the lesions and pain may limit physical activities. The prognosis was assessed in 27 patients with longstanding Disease and the long-term outlook is generally good. Seventeen patients had improved and the Disease was static in seven patients. Three patients deteriorated with age. Topical corticosteroids with or without added antibiotics were an effective treatment.
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Seborrhoeic dermatitis of the scalp--a manifestation of Hailey-Hailey Disease in a predisposed individual?
The British journal of dermatology, 1992Co-Authors: P. Marren, S.m. BurgeAbstract:Summary A 59-year-old man was found to have typical Hailey–Hailey Disease of the back, neck and axillae. In addition, he had fine white scaling in the scalp and postanricular areas. Despite the clinical appearance of seborrhoeic dermatitis, a biopsy of his scalp showed prominent suprabasal epidermal separation with acantholysis. We propose that in a genetically predisposed individual. Hailey–Hailey Disease can assume atypical and non-specific appearances.
Amor Khachemoune - One of the best experts on this subject based on the ideXlab platform.
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Hailey–Hailey Disease: An Update Review with a Focus on Treatment Data
American Journal of Clinical Dermatology, 2019Co-Authors: Imene Ben Lagha, Kurt Ashack, Amor KhachemouneAbstract:Hailey–Hailey Disease is a rare blistering dermatosis first described in 1939 by the brothers Howard and Hugh Hailey. Its incidence is estimated at 1/50,000. The inheritance is autosomal dominant with complete penetrance, but a variable expressivity in affected family members. Clinically, Hailey–Hailey Disease presents between the third and fourth decade as flaccid vesicles and blisters on erythematous skin, giving rise to erosions, fissures, and vegetations. Maceration and superinfections are frequent. The lesions are typically distributed symmetrically within intertriginous regions such as the retroauricular folds, lateral aspects of the neck, axillae, umbilicus, inguinal, and perianal regions. The Disease is characterized by a chronic relapsing course with spontaneous remissions and multiple recurrences. Severe Disease can be very frustrating and have a major psychological and social impact. Given the dearth of evidence-based guidelines and large clinical trials, the assessment of the efficacy and safety of treatments is difficult. Treatments include topical and systemic agents, and procedural therapy such as lasers and surgery. This review provides a systematic search of the literature with a focus on classical and emerging treatment options for Hailey–Hailey Disease.
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Hailey-Hailey Disease: An Update Review with a Focus on Treatment Data.
American journal of clinical dermatology, 2019Co-Authors: Imene Ben Lagha, Kurt Ashack, Amor KhachemouneAbstract:Hailey–Hailey Disease is a rare blistering dermatosis first described in 1939 by the brothers Howard and Hugh Hailey. Its incidence is estimated at 1/50,000. The inheritance is autosomal dominant with complete penetrance, but a variable expressivity in affected family members. Clinically, Hailey–Hailey Disease presents between the third and fourth decade as flaccid vesicles and blisters on erythematous skin, giving rise to erosions, fissures, and vegetations. Maceration and superinfections are frequent. The lesions are typically distributed symmetrically within intertriginous regions such as the retroauricular folds, lateral aspects of the neck, axillae, umbilicus, inguinal, and perianal regions. The Disease is characterized by a chronic relapsing course with spontaneous remissions and multiple recurrences. Severe Disease can be very frustrating and have a major psychological and social impact. Given the dearth of evidence-based guidelines and large clinical trials, the assessment of the efficacy and safety of treatments is difficult. Treatments include topical and systemic agents, and procedural therapy such as lasers and surgery. This review provides a systematic search of the literature with a focus on classical and emerging treatment options for Hailey–Hailey Disease.
Rudolf Happle - One of the best experts on this subject based on the ideXlab platform.
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segmental Hailey Hailey Disease of the vulva
Pediatric Dermatology, 2018Co-Authors: Irene Garciamorales, Rudolf Happle, Luis Requenacaballero, Antonio TorreloAbstract:: A female infant presented with an ulcerated lesion on the right side of the vulva. Histopathology showed a suprabasal acantholytic blister with intact papillae protruding into the blister cavity and a few dyskeratotic cells. There were no signs of injury on other locations. Family history was unremarkable. Our patient may have linear Hailey-Hailey Disease of the vulva, most likely a case of type 1 mosaic.
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Type 2 segmental Hailey-Hailey Disease with systematized bilateral arrangement.
International journal of dermatology, 2013Co-Authors: Arti Nanda, Fatima Khawaja, Humoud Al-sabah, Rudolf HappleAbstract:Hailey–Hailey Disease (HHD) is an autosomal dominantdisorder characterized by recurrent vesicles and erosionspredominantly of intertriginous areas. Lesions usuallyappear after puberty and continue throughout life. Histo-pathologically, acantholysis with epidermal clefting isnoted. Widespread partial loss of intercellular bridgesbetween the keratinocytes gives the epidermis anappearance resembling that of a dilapidated brick wall.
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Treatment of Hailey-Hailey Disease by dermabrasion
Journal of the American Academy of Dermatology, 1993Co-Authors: Gudula Kirtschig, Uwe Gieler, Rudolf HappleAbstract:Hailey-Hailey Disease (benign familial chronic pemphigus) is an autosomal dominant disorder characterized by erosions, blisters, and warty papules localized mainly in intertriginous areas. The Disease has a variable course with periods of exacerbation that can be suppressed by topical glucocorticosteroids and antiseptics. However, in recalcitrant cases, surgical procedures appear to be effective. Several authors have reported favorable results obtained by excision of involved skin with subsequent grafting of the defect. 1-5 In 1983 Belhaouari et a1.6 and in 1989 Hamm? described successful dermabrasion in cases of Hailey-Hailey Disease resistant to conventional therapy. We report the cases of two patients in whom dermabrasion led to a long-standing absence of skin lesions.
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Leukonychia longitudinalis as the primary symptom of Hailey-Hailey Disease
Hautarzt, 1992Co-Authors: Gudula Kirtschig, Isaak Effendy, Rudolf HappleAbstract:: Longitudinal white bands on the fingernails were observed in a 58-year-old male patient with perianal intertrigo. His nail changes were a first clue to the diagnosis of Hailey-Hailey Disease, which was later confirmed histopathologically. Similar nail abnormalities were found in the patient's son who was also affected with Hailey-Hailey Disease. These findings support the concept that those nail changes, first described by Burge in 1991, constitute a useful diagnostic marker in Hailey-Hailey Disease.
Kurt Ashack - One of the best experts on this subject based on the ideXlab platform.
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Hailey–Hailey Disease: An Update Review with a Focus on Treatment Data
American Journal of Clinical Dermatology, 2019Co-Authors: Imene Ben Lagha, Kurt Ashack, Amor KhachemouneAbstract:Hailey–Hailey Disease is a rare blistering dermatosis first described in 1939 by the brothers Howard and Hugh Hailey. Its incidence is estimated at 1/50,000. The inheritance is autosomal dominant with complete penetrance, but a variable expressivity in affected family members. Clinically, Hailey–Hailey Disease presents between the third and fourth decade as flaccid vesicles and blisters on erythematous skin, giving rise to erosions, fissures, and vegetations. Maceration and superinfections are frequent. The lesions are typically distributed symmetrically within intertriginous regions such as the retroauricular folds, lateral aspects of the neck, axillae, umbilicus, inguinal, and perianal regions. The Disease is characterized by a chronic relapsing course with spontaneous remissions and multiple recurrences. Severe Disease can be very frustrating and have a major psychological and social impact. Given the dearth of evidence-based guidelines and large clinical trials, the assessment of the efficacy and safety of treatments is difficult. Treatments include topical and systemic agents, and procedural therapy such as lasers and surgery. This review provides a systematic search of the literature with a focus on classical and emerging treatment options for Hailey–Hailey Disease.
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Hailey-Hailey Disease: An Update Review with a Focus on Treatment Data.
American journal of clinical dermatology, 2019Co-Authors: Imene Ben Lagha, Kurt Ashack, Amor KhachemouneAbstract:Hailey–Hailey Disease is a rare blistering dermatosis first described in 1939 by the brothers Howard and Hugh Hailey. Its incidence is estimated at 1/50,000. The inheritance is autosomal dominant with complete penetrance, but a variable expressivity in affected family members. Clinically, Hailey–Hailey Disease presents between the third and fourth decade as flaccid vesicles and blisters on erythematous skin, giving rise to erosions, fissures, and vegetations. Maceration and superinfections are frequent. The lesions are typically distributed symmetrically within intertriginous regions such as the retroauricular folds, lateral aspects of the neck, axillae, umbilicus, inguinal, and perianal regions. The Disease is characterized by a chronic relapsing course with spontaneous remissions and multiple recurrences. Severe Disease can be very frustrating and have a major psychological and social impact. Given the dearth of evidence-based guidelines and large clinical trials, the assessment of the efficacy and safety of treatments is difficult. Treatments include topical and systemic agents, and procedural therapy such as lasers and surgery. This review provides a systematic search of the literature with a focus on classical and emerging treatment options for Hailey–Hailey Disease.
S E Thomas - One of the best experts on this subject based on the ideXlab platform.
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squamous cell carcinoma arising in Hailey Hailey Disease of the vulva
British Journal of Dermatology, 2000Co-Authors: S E Cockayne, D M Rassl, S E ThomasAbstract:A 61-year-old woman, who was known to have Hailey-Hailey Disease, presented with increasing vulval soreness. Biopsy showed vulval intraepithelial neoplasia (VIN) 3 and subsequent histology from a vulvectomy specimen showed extensive VIN with early invasive squamous cell carcinoma. This may be another example of chronic inflammation of the vulval area leading to the development of squamous cell carcinoma. However, in this case, chronic human papillomavirus may also have played a part, leading to VIN and reactivation of the Hailey-Hailey Disease. We can find no previous reports of squamous cell carcinoma developing in the setting of Hailey-Hailey Disease.
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Squamous cell carcinoma arising in Hailey–Hailey Disease of the vulva
The British journal of dermatology, 2000Co-Authors: S E Cockayne, D M Rassl, S E ThomasAbstract:A 61-year-old woman, who was known to have Hailey-Hailey Disease, presented with increasing vulval soreness. Biopsy showed vulval intraepithelial neoplasia (VIN) 3 and subsequent histology from a vulvectomy specimen showed extensive VIN with early invasive squamous cell carcinoma. This may be another example of chronic inflammation of the vulval area leading to the development of squamous cell carcinoma. However, in this case, chronic human papillomavirus may also have played a part, leading to VIN and reactivation of the Hailey-Hailey Disease. We can find no previous reports of squamous cell carcinoma developing in the setting of Hailey-Hailey Disease.
