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Papers of the Week

2021 Jan 28

Orphanet J Rare Dis



Release of pseudosyndactyly in recessive dystrophic epidermolysis bullosa using a dermal regeneration template glove: the Foggia experience.


Lembo F, Parisi D, Cecchino L R, Ciancio F, Innocenti A, Portincasa A
Orphanet J Rare Dis. 2021 Jan 28; 16(1):52.
PMID: 33509245.


Epidermolysis bullosa (EB) comprises a heterogeneous group of rare genetic diseases associated with skin blistering caused by minimal trauma. A major and common EB subtype, recessive dystrophic EB (RDEB), is characterized by altered wound healing, inflammatory dysbalance and fibrotic changes associated with reduced to absent collagen VII. Because of its exposed position and its continued use in daily activities, the hand is constantly at risk of microtrauma and is therefore one of the organs most affected by the disease with highly disabling deformities that represent a challenging field in hand surgery practice.