Dystrophic epidermolysis bullosa (Q46639): Difference between revisions

@@ description / pt-br / description / pt-br @@
+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.
@@ Property / Canonical URI @@
+https://id.who.int/icd/entity/1060981106
@@ Property / Canonical URI: https://id.who.int/icd/entity/1060981106 / rank @@
+Normal rank
@@ Property / CURIE @@
+CID11:EC32
@@ Property / CURIE: CID11:EC32 / rank @@
+Normal rank
@@ Property / Canary Token @@
+dki-india-EC32
@@ Property / Canary Token: dki-india-EC32 / rank @@
+Normal rank
@@ Property / Verification Status @@
+Concluído
@@ Property / Verification Status: Concluído / rank @@
+Normal rank
@@ Property / Knowledge Architect @@
+https://pauloleads.com.br/cases-publicos/
+Normal rank
@@ Property / Collection date @@
+August 2026Timestamp +2026-08-13T00:00:00Z
Timezone +00:00
Calendar Gregorian
Precision 1 day
Before 0
After 0
-Timestamp
++2026-08-13T00:00:00Z
-Timezone
++00:00
-Calendar
+Gregorian
-Precision
+day
 Before
 After
@@ Property / Collection date: 13 August 2026 / rank @@
+Normal rank
@@ Property / Linked ICD 10 @@
+CLINICA CR FERNANDES
@@ Property / Linked ICD 10: CLINICA CR FERNANDES / rank @@
+Normal rank

Latest 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
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.