Epidermolysis bullosa pruriginosa.
Epidermolysis bullosa (EB) pruriginosa is a rare clinical subset of dystrophic EB, characterized by marked itching and presence of prurigo-like or lichenoid features. In order to further delineate the phenotype and understand the pathogenesis of this disorder, the clinical, histological and ultrastructural findings of a 19-year-old patient presenting a typical form of EB pruriginosa are described. The prevalence of papular itchy lichenoid lesions, signs of scratching and paucity of blisters at the time of clinical examination may result in incorrect diagnosis and treatment. Microscopic studies of the lesions show the typical findings of dystrophic EB associated with an unusually high density of collagen bundles and absence of elastic fibres in the upper dermis. Itching lichenoid lesions of EB pruriginosa could represent an abnormal dermal reactivity of some patients to their inherited bullous disorder.
Cambiaghi
S
S
Centre for Inherited Skin Disorders, IRCCS Ospedale Maggiore, University of Milan, Italy.
Brusasco
A
A
Restano
L
L
Cavalli
R
R
Tadini
G
G
D000328:Adult; D001768:Blister / Q000473:pathology; D002921:Cicatrix / Q000473:pathology; D003094:Collagen / Q000648:ultrastructure; D004547:Elastic Tissue / Q000473:pathology / Q000648:ultrastructure; D004814:Epidermal Cyst / Q000473:pathology / Q000648:ultrastructure; D016108:Epidermolysis Bullosa Dystrophica / Q000209:etiology / Q000473:pathology*; D005260:Female; D006801:Humans; D017512:Lichenoid Eruptions / Q000209:etiology / Q000473:pathology*; D009260:Nail Diseases / Q000473:pathology; D010641:Phenotype; D015995:Prevalence; D011536:Prurigo / Q000209:etiology / Q000473:pathology*; D011537:Pruritus; D012867:Skin / Q000473:pathology / Q000648:ultrastructure
D003094:Collagen