Dystrophic epidermolysis bullosa (Q46639): Difference between revisions
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A epidermólise bolhosa distrófica (dermolítica) é devida a defeitos ou ausência de colágeno tipo VII. Como resultado, as fibrilas de ancoragem que fixam a membrana basal epidérmica à derme são defeituosas ou ausentes. A forma recessiva grave causa cicatrizes extensas e predispõe ao carcinoma escamoso (espinocelular) agressivo. | |||
| description / en | description / en | ||
Dystrophic (dermolytic) epidermolysis bullosa is due to defects in or absence of type VII collagen. As a result the anchoring fibrils which secure the epidermal basement membrane to the dermis are defective or absent. The severe recessive form causes extensive scarring and predisposes to aggressive squamous cell carcinoma. | |||
Revision as of 15:21, 13 August 2026
Dystrophic (dermolytic) epidermolysis bullosa is due to defects in or absence of type VII collagen. As a result the anchoring fibrils which secure the epidermal basement membrane to the dermis are defective or absent. The severe recessive form causes extensive scarring and predisposes to aggressive squamous cell carcinoma.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | EC32 |
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| English | Dystrophic epidermolysis bullosa |
Dystrophic (dermolytic) epidermolysis bullosa is due to defects in or absence of type VII collagen. As a result the anchoring fibrils which secure the epidermal basement membrane to the dermis are defective or absent. The severe recessive form causes extensive scarring and predisposes to aggressive squamous cell carcinoma. |
