Epidermolysis bullosa and the risk of life-threatening cancers: The National EB Registry experience, 1986-2006

Fine, Jo David; Johnson, Lorraine; Weiner, Madeline; Li, Kuo Ping; Suchindran, Chirayath

doi:10.1016/j.jaad.2008.09.035

Cited by 357 publications

(356 citation statements)

References 39 publications

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“…The disruption of this complex leads to a weak dermalepidermal junction and the epidermis can easily separates from the dermis leading to debilitating widespread blistering. This condition is complicated by the subsequent development of aggressive and often metastatic cSCC (Fine, Johnson et al 2009). …”

Section: Extracellular Matrix Can Profoundly Influence Tumour Progresmentioning

confidence: 99%

Tumour–stroma crosstalk in the development of squamous cell carcinoma

Lim

South

2014

The International Journal of Biochemistry & Cell Biology

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Section: Extracellular Matrix Can Profoundly Influence Tumour Progresmentioning

confidence: 99%

Tumour–stroma crosstalk in the development of squamous cell carcinoma

Lim

South

2014

The International Journal of Biochemistry & Cell Biology

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“…Mechanically induced skin blistering, painful wounds, and healing with scarring severely impede the quality of life of the affected individuals (2,3). Injury-derived progressive soft tissue fibrosis drives formation of aggressive squamous cell carcinoma, which is the main cause of death in severe RDEB (4,5). Collagen VII has been viewed as a rather skin-specialized protein, but extracutaneous expression also occurs (6).…”

mentioning

confidence: 99%

Impaired lymphoid extracellular matrix impedes antibacterial immunity in epidermolysis bullosa

Nyström

Bornert

Kühl

et al. 2018

Proc. Natl. Acad. Sci. U.S.A.

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Genetic loss of collagen VII causes recessive dystrophic epidermolysis bullosa (RDEB), a skin fragility disorder that, unexpectedly, manifests also with elevated colonization of commensal bacteria and frequent wound infections. Here, we describe an unprecedented systemic function of collagen VII as a member of a unique innate immune-supporting multiprotein complex in spleen and lymph nodes. In this complex, collagen VII specifically binds and sequesters the innate immune activator cochlin in the lumen of lymphoid conduits. In genetic mouse models, loss of collagen VII increased bacterial colonization by diminishing levels of circulating cochlin LCCL domain. Intraperitoneal injection of collagen VII, which restored cochlin in the spleen, but not in the skin, reactivated peripheral innate immune cells via cochlin and reduced bacterial skin colonization. Systemic administration of the cochlin LCCL domain was alone sufficient to diminish bacterial supercolonization of RDEB mouse skin. Human validation demonstrated that RDEB patients displayed lower levels of systemic cochlin LCCL domain with subsequently impaired macrophage response in infected wounds. This study identifies an intrinsic innate immune dysfunction in RDEB and uncovers a unique role of the lymphoid extracellular matrix in systemic defense against bacteria.collagen VII | innate immunity | bacteria | recessive dystrophic epidermolysis bullosa | cochlin

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“…En sık RDEB' da ancak DDEB ve JEB'da da görülebilir. Risk genellikle 2. dekatta başlar, agresif seyirli ve kötü prognozludur [28][29][30][31][32][33][34][35][36][37][38] .…”

Section: Deri Dışı Komplikasyonlarunclassified

Inherited Bullous Diseases

Karaduman¹

2011

Turkderm

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ÖzetEpidermolizis bülloza(EB), deri ve mukozaların frajilitesi ve küçük travmalarla bile bül oluşumu ile karakterize bir grup kalıtsal büllöz hastalığı içerir. Deri frajilitesi ve bül formasyonu epidermis, dermo-epidermal bileşke ve üst dermisde normalde de bulunan çok sayıda proteini kodlayan genlerde mutasyon sonucu oluşur. Epidermolizis bülloza simpleks (EBS), Junktional EB (JEB), distrofik EB ( Sum maryEpidermolysis bullosa (EB), refers to a group of inherited bullous disorders, characterized by fagility of the skin and mucous membranes, and blister formation in response to minor friction or trauma. Skin fragility and bulla formation of EB result from genetic mutations of any of dozen genes that encode structural proteins which normally reside within the epidermis, the dermo-epidermal juntion, or the upper dermis. There are four major type of inherited epidermolysis bullosa: EB simplex (EBS), junctional EB (JEB), dystrophic EB (

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Epidermolysis bullosa and the risk of life-threatening cancers: The National EB Registry experience, 1986-2006

Cited by 357 publications

References 39 publications

Tumour–stroma crosstalk in the development of squamous cell carcinoma

Tumour–stroma crosstalk in the development of squamous cell carcinoma

Impaired lymphoid extracellular matrix impedes antibacterial immunity in epidermolysis bullosa

Inherited Bullous Diseases

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