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Case report of a novel association between anti-p200 pemphigoid and acquired haemophilia A
Xuan Qi Koh,Kong Bing Tan,Enno Schmidt,Detlef Zillikens,Nisha Suyien Chandran 대한피부과학회 2023 Annals of Dermatology Vol.35 No.1
Anti-p200 pemphigoid is an uncommon subepidermal autoimmune bullous disease that,unlike many other autoimmune bullous diseases, has not previously been associated withhematological diseases. The diagnosis of anti-p200 pemphigoid in a patient with congruentclinical features requires the demonstration of subepidermal blistering, with linear depositionof immunoglobulin (Ig) G and/or C3 at the dermoepidermal junction on direct immunofluorescence,and a floor-binding pattern on indirect immunofluorescence. In addition,the detection of antibodies against p200 antigen via immunoblotting is ideal but not readilyaccessible in many facilities, leading to a potential under-recognition and under-diagnosisof this condition. In this case report, we describe a 53-year-old gentleman with recently diagnosedacquired hemophilia A who developed a concurrent vesiculobullous eruption andwas evaluated to have anti-p200 pemphigoid. Both of his conditions were controlled withimmunosuppression via prednisolone and cyclophosphamide. While we acknowledge thecontemporaneous occurrence of both diseases in this patient may be a mere coincidence, it isimportant to recognize the possibility of this association given the potential clinical significance. Whether the activity of one disease parallels the other will require further evaluation.