Faculty Opinions recommendation of Loss of corneodesmosin leads to severe skin barrier defect, pruritus, and atopy: unraveling the peeling skin disease.

Author(s):  
Akemi Ishida-Yamamoto
2010 ◽  
Vol 87 (2) ◽  
pp. 274-281 ◽  
Author(s):  
Vinzenz Oji ◽  
Katja-Martina Eckl ◽  
Karin Aufenvenne ◽  
Marc Nätebus ◽  
Tatjana Tarinski ◽  
...  

2014 ◽  
Vol 395 (9) ◽  
pp. 945-958 ◽  
Author(s):  
Laetitia Furio ◽  
Alain Hovnanian

Abstract Netherton syndrome (NS) is an orphan genetic skin disease with a profound skin barrier defect and severe allergic manifestations. NS is caused by loss of function mutations in SPINK5 encoding lympho-epithelial Kazal-type inhibitor (LEKTI), a secreted multi-domain serine protease inhibitor expressed in stratified epithelia. Studies in mouse models and in NS patients have established that unopposed kallikrein 5 activity triggers stratum corneum detachment and activates PAR-2 signaling, leading to the autonomous production of pro-allergic and pro-inflammatory mediators. This emerging knowledge on NS pathogenesis has highlighted a central role for protease regulation in skin homeostasis but also in the complexity of the disease, and holds the promise of new specific treatments.


2014 ◽  
Vol 134 (1) ◽  
pp. 105-111 ◽  
Author(s):  
Peter Bognar ◽  
Ilona Nemeth ◽  
Balazs Mayer ◽  
Dora Haluszka ◽  
Norbert Wikonkal ◽  
...  

2021 ◽  
pp. e2021091
Author(s):  
Rachel Graubard ◽  
Ariadna Perez-Sanchez ◽  
Rajani Katta

Stress has multiple and wide-ranging physiologic and clinical impacts on skin disease. This has led to an interest in mind body therapies as potential adjunct treatments for skin disease. The stress response results in the activation of the endocrine, neurologic, and immune systems, with a resulting cascade of impacts, that are both systemic and cutaneous. The 2 main arms of the stress response are the sympathetic nervous system and the hypothalamic-pituitary-adrenal axis. The resultant release of cortisol, catecholamines, and neuropeptides has multiple effects. Clinically, these have been shown to increase skin inflammation, increase itching, impair skin barrier function, impair wound healing, and suppress immunity.Mind body therapies are those that focus on the interaction between the mind and the body, with the goal to influence physical function and impact health. These have been shown to ameliorate some of the harmful physiologic changes attributed to stress or to reduce harmful behaviors. In some cases, such as with biofeedback, they may also result in beneficial physiologic changes. Treatments such as meditation, biofeedback, hypnosis, guided imagery, and others have been evaluated in the treatment of skin disease and have shown some benefits. Although randomized controlled trials are limited, these interventions have shown beneficial effects on itching, psychosocial outcomes, and even skin severity. These interventions have been evaluated in diseases such as atopic dermatitis, psoriasis, trichotillomania, and others. Given the potential benefits, improvements in psychosocial outcomes, and a low risk profile, referral to qualified practitioners or multidisciplinary clinics should be considered for some patients.


F1000Research ◽  
2015 ◽  
Vol 4 ◽  
pp. 1296 ◽  
Author(s):  
Michael Barton ◽  
Robert Sidbury

Atopic dermatitis is a chronic, pruritic skin disease characterized by an improperly functioning skin barrier and immune dysregulation. We review proposed atopic dermatitis pathomechanisms, emphasizing how these impact current perspectives on natural history, role of allergic sensitization, and future therapeutic targets.


2019 ◽  
Vol 4 (3) ◽  
pp. 01-08
Author(s):  
Nora Harfouch ◽  
Fouz Hassan

Background:Atopic dermatitis (AD) is a common chronic inflammatory skin disorder that induces several symptoms including pruritus and dryness, and is often associated with secondary cutaneous infections. AD is considered to be one of the most prevalent and studied skin diseases yet poorly understood, and its pathophysiology remains obscure. Even though other skin diseases (such as psoriasis) share the same pathologic factor -skin barrier defect - with atopic dermatitis, patients diagnosed with those diseases don't suffer infectious exacerbations like atopic patients do. Aim: Although many international researches have already discussed the relationship between staphylococcus aureus and AD, no studies about this subject in the Arabic region was documented. The aim of our study is to compare staphylococcus aureus colonization rates and densities between atopic dermatitis patients and non-atopic subjects, and to relate the colonization to the severity and duration of the disease. Materials and methods: This observational analytic study included 200 participants (99 diagnosed with atopic dermatitis and 101 control subjects without atopic dermatitis); nasal and skin swabs (lesional and non-lesional) were collected from patients, while nasal and only normal skin swabs were collected from controls. Positive swabs were assessed to determine the density of colonization. Results: 57.6% of patients had nasal colonization, 56.6% had lesional colonization and 30.3% had normal skin colonization. Nasal colonization rates and densities were higher in the patients group. We detected a correlation between colonization and severity of eczema, but no correlation between colonization and duration of the disease was detected. Conclusion: The high rates and densities of staphylococcus aureus colonization in lesional skin of atopic dermatitis patients point out the role of these organisms in the pathophysiology of the disease, put antibiotics on the treatment list of atopic dermatitis and explain infectious features in AD exacerbations.


2009 ◽  
Vol 285 (10) ◽  
pp. 7300-7311 ◽  
Author(s):  
Franz P. W. Radner ◽  
Ingo E. Streith ◽  
Gabriele Schoiswohl ◽  
Martina Schweiger ◽  
Manju Kumari ◽  
...  

2016 ◽  
Vol 136 (9) ◽  
pp. S182
Author(s):  
S. Danby ◽  
H. Wan ◽  
J. Chittock ◽  
K. Brown ◽  
A. Wigley ◽  
...  

2020 ◽  
Vol 8 (11) ◽  
pp. 1743 ◽  
Author(s):  
Krzysztof Szalus ◽  
Magdalena Trzeciak ◽  
Roman J. Nowicki

A common disease worldwide is known as atopic dermatitis (AD), named also as atopic eczema, which is a chronic recurrent complex inflammatory skin disorder. It affects 2–10% of the adult population and up to 20% of the pediatric population. The clinical AD picture appears in typically localized eczema and dry skin, and is dominated by a persistent pruritus followed by sleep disturbances. AD strongly impacts on the quality of life of AD patients and their families as well as on social and economic aspects. The pathogenesis of the disease is complex and consists of multiple interactions between immunological disturbances, skin barrier defect, and microbial dysbiosis with environmental influences. The treatment of AD reflects the pathogenetic disorders, starting from basic emollient therapy, and goes to topical anti-inflammatory regimens followed by phototherapy, systemic immunosuppressive drugs, and new biologic immunomodulators. This paper will thus summarize the novel collection of biological treatment JAK-STAT inhibitors dedicated to AD.


2001 ◽  
Vol 155 (5) ◽  
pp. 821-832 ◽  
Author(s):  
Martyn Chidgey ◽  
Cord Brakebusch ◽  
Erika Gustafsson ◽  
Alan Cruchley ◽  
Chris Hail ◽  
...  

The desmosomal cadherin desmocollin (Dsc)1 is expressed in upper epidermis where strong adhesion is required. To investigate its role in vivo, we have genetically engineered mice with a targeted disruption in the Dsc1 gene. Soon after birth, null mice exhibit flaky skin and a striking punctate epidermal barrier defect. The epidermis is fragile, and acantholysis in the granular layer generates localized lesions, compromising skin barrier function. Neutrophils accumulate in the lesions and further degrade the tissue, causing sloughing (flaking) of lesional epidermis, but rapid wound healing prevents the formation of overt lesions. Null epidermis is hyperproliferative and overexpresses keratins 6 and 16, indicating abnormal differentiation. From 6 wk, null mice develop ulcerating lesions resembling chronic dermatitis. We speculate that ulceration occurs after acantholysis in the fragile epidermis because environmental insults are more stringent and wound healing is less rapid than in neonatal mice. This dermatitis is accompanied by localized hair loss associated with formation of utriculi and dermal cysts, denoting hair follicle degeneration. Possible resemblance of the lesions to human blistering diseases is discussed. These results show that Dsc1 is required for strong adhesion and barrier maintenance in epidermis and contributes to epidermal differentiation.


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