Case report of a novel association between anti-p200 pemphigoid and acquired haemophilia A
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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.
Abstract
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.
참고문헌 (15)
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