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Orphanet J Rare Dis ; 8: 69, 2013 May 07.
Article in English | MEDLINE | ID: mdl-23651789

ABSTRACT

Blistering in epidermolysis bullosa simplex type Dowling-Meara (EBS-DM) is associated with an inflammatory phenotype, which can be disrupted by diacerein in vitro. In this pilot study we hypothesized, that a topical formulation of diacerein 1% reduces blistering. Five patients initially applied diacerein underneath both armpits. Then, each participant received 1% diacerein-cream for one armpit, and placebo for the other (randomized withdrawal). The number of blisters was reduced significantly (left: -78%; right: -66% of baseline) within two weeks and remained significantly below the initial level even during withdrawal in four patients. These findings point to a relevant effect of diacerein and provide important information for a confirmative study.


Subject(s)
Anthraquinones , Anti-Inflammatory Agents , Epidermolysis Bullosa Simplex/drug therapy , Administration, Topical , Adolescent , Adult , Anthraquinones/administration & dosage , Anthraquinones/therapeutic use , Anti-Inflammatory Agents/administration & dosage , Anti-Inflammatory Agents/therapeutic use , Blister/drug therapy , Child , Double-Blind Method , Epidermolysis Bullosa Simplex/genetics , Epidermolysis Bullosa Simplex/pathology , Humans , Keratin-14/genetics , Middle Aged , Pilot Projects , Treatment Outcome , Young Adult
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