Case report of a novel association between anti-p200 pemphigoid and acquired haemophilia A

다국어 초록 (Multilingual 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 (Reference)

1 Binet Q, "Successful management of acquired hemophilia A associated with bullous pemphigoid : a case report and review of the literature" 2017 : 2057019-, 2017

2 Seo SH, "Pharyngeal obstruction due to hemorrhagic bullae in a patient with anti-BP180-type mucous membrane pemphigoid associated with acquired hemophilia A" 46 : e375-e376, 2019

3 Tiede A, "International recommendations on the diagnosis and treatment of acquired hemophilia A" 105 : 1791-1801, 2020

4 Knoebl P, "Demographic and clinical data in acquired hemophilia A : results from the European Acquired Haemophilia Registry(EACH2)" 10 : 622-631, 2012

5 Filipczak A, "Coexistence of pemphigus foliaceus and acquired hemophilia A : a case report" 42 : 638-641, 2015

6 Yan TM, "Coexistence of acquired hemophilia A and epidermolysis bullosa acquisita : two case reports and published work review" 44 : 76-79, 2017

7 Arakaki O, "Case of linear immunoglobulin A bullous dermatosis associated with acquired hemophilia" 35 : 437-446, 2008

8 Abdul-Halim NA, "Bullous pemphigoid is a common associated disorder with acquired haemophilia A" 113 : 58-62, 2021

9 Fakprapai W, "Bullous pemphigoid associated with acquired hemophilia A : a case report and review of the literature" 11 : 130-139, 2019

10 Nishiura N, "Autoimmune bullous disease and Hashimoto's disease complicated by acquired hemophilia A" 58 : 233-238, 2017

11 Lau I, "Anti-p200 pemphigoid is the most common pemphigoid disease with serum antibodies against the dermal side by indirect immunofluorescence microscopy on human salt-split skin" 81 : 1195-1197, 2019

12 Kridin K, "Anti-p200 pemphigoid : a systematic review" 10 : 2466-, 2019

13 Goletz S, "Anti-p200 pemphigoid" 71 : 185-191, 2014

14 Ly A, "Anti-laminin 5 pemphigoid and acquired haemophilia" 146 : 1104-1105, 2002

15 Chan AY, "A novel human autoimmune syndrome caused by combined hypomorphic and activating mutations in ZAP-70" 213 : 155-165, 2016