返回Google圖書搜尋

Hyperkeratotic Bullous Pemphigoid: 5 Cases of a New Clinical Subset

出版	Morressier, 2017
URL	http://books.google.com.hk/books?id=4A-2zQEACAAJ&hl=&source=gbs_api

註釋BackgroundThe clinical features of bullous pemphigoid are extremely polymorphous and its cutaneous manifestations protean. Several atypical forms of bullous pemphigoid have been described, and the diagnosis critically relies on immunopathologic findings. ObjectiveThe purpose of the study was to describe a new variant of bullous pemphigoid characterized by diffuse hyperkeratosis and palmo-plantar keratoderma with high IgE levels, that we named hyperkeratotic bullous pemphigoid.MethodsWe performed a case-series study. Five patients fulfilled the diagnostic criteria of bullous pemphigoid. We evaluated the severity of the disease using the ABSIS score. We used commercial ELISAs to detect IgG and IgE anti-BP180 and anti-BP230 antibodies.ResultsWe described five hyperkeratotic bullous pemphigoid patients with palmo-plantar keratoderma, diffuse hyperkeratotic cutaneous lesions, and extremely high total IgE levels. Some of these patients presented a resistant bullous pemphigoid, which required multiple systemic treatments.ConclusionThe diagnosis of hyperkeratotic bullous pemphigoid should be taken into account in patients presenting diffuse hyperkeratotic cutaneous lesions and palmo-plantar keratoderma, even in the absence of blisters, as this clinical variant is likely to pose additional challenges in the choice of an effective treatment regimen.