Thalidomide in the management of epidermolysis bullosa pruriginosa.
Epidermolysis bullosa (EB) pruriginosa is a distinctive clinical subtype of dystrophic EB. We report a patient with dominant dystrophic EB pruriginosa, who had an excellent response to systemic thalidomide treatment. The mechanism of action of thalidomide in the management of pruriginous disorders is not yet completely understood. Most recent studies point towards an immunomodulatory action of thalidomide that may suppress excessive production of tumour necrosis factor-alpha and may downregulate certain cell surface adhesion molecules involved in leucocyte migration.
Ozanic Bulic
S
S
Department of Dermatology, Great Ormond Street Hospital for Children, London WC1N 3JH, UK.
Fassihi
H
H
Mellerio
J E
JE
McGrath
J A
JA
Atherton
D J
DJ
D000293:Adolescent; D016108:Epidermolysis Bullosa Dystrophica / Q000188:drug therapy* / Q000235:genetics; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D017354:Point Mutation; D011536:Prurigo / Q000188:drug therapy / Q000235:genetics; D013792:Thalidomide / Q000627:therapeutic use*
D007166:Immunosuppressive Agents; D013792:Thalidomide