Acquired Hemophilia A associated with Bullous Pemphigoid: a rare combination

Background. Acquired hemophilia A (AHA) is a rare condition, due to the spontaneous formation of neutralizing antibodies against endogenous factor VIII. About half the cases are associated with pregnancy, postpartum, autoimmune diseases, malignancies, or adverse drug reactions. Symptoms include severe and unexpected bleeding that may prove life-threatening.Case Study. We report a case of AHA associated with bullous pemphigoid (BP), a chronic, autoimmune, subepidermal, blistering skin disease. To our knowledge, this is the 25th documented case of such an association. Following treatment for less than 3 months consisting of methylprednisolone at decreasing dose levels along with four courses of rituximab (monoclonal antibody directed against the CD20 protein), AHA was completely cured and BP well-controlled.Conclusions. This report illustrates a rare association of AHA and BP, supporting the possibility of eradicating the inhibitor with a well-conducted short-term treatment.

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Acquired hemophilia A associated with bullous pemphigoid and multiple myeloma: a case report

Annales de biologie clinique ◽

10.1684/abc.2018.1405 ◽

2019 ◽

Vol 77 (2) ◽

pp. 179-183 ◽

Cited By ~ 1

Author(s):

Élise Sourdeau ◽

Sylvain Clauser ◽

Romain Prud’Homme ◽

Valérie Bardet ◽

Leyla Calmette

Keyword(s):

Multiple Myeloma ◽

Case Report ◽

Bullous Pemphigoid ◽

Hemophilia A ◽

Acquired Hemophilia A ◽

Acquired Hemophilia

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Diagnosis dan Tata Laksana Acquired Hemophilia A (AHA) dengan Pemfigoid Bulosa

Jurnal Penyakit Dalam Indonesia ◽

10.7454/jpdi.v3i4.56 ◽

2017 ◽

Vol 3 (4) ◽

pp. 218

Author(s):

Rasco Sandy Sihombing ◽

Henry Ratno Diono Silalahi ◽

Hamzah Shatri ◽

Lugyanti Sukrisman ◽

Ikhwan Rinaldi ◽

...

Keyword(s):

Case Report ◽

Factor Viii ◽

Bullous Pemphigoid ◽

Skin Disorder ◽

Hemophilia A ◽

Coagulation Factor ◽

Acquired Hemophilia A ◽

Acquired Hemophilia ◽

Faktor Viii ◽

Autoantibody Formation

Acquired hemophilia A merupakan kondisi dimana faktor koagulasi VIII menjadi tidak aktif akibat pembentukan autoantibodi. Kondisi ini dikaitkan dengan kehamilan, keganasan, dan penyakit auitoimun dengan kelainan kulit. Pada kasus ini, seorang wanita berusia 66 tahun, datang dengan keluhan perdarahan paska tindakan yang disertai dengan lesi kulit. Pasien didiagnosis dengan acquired hemophilia A dengan ditemukannya inhibitor faktor VIII terkait dengan pemfigoid bulosa.Kata Kunci: Acquired hemophilia A, diagnosis, pemfigoid bulosa, tata laksana Diagnosis and Treatment of Acquired Hemophilia A (AHA) with Bullous PemphigoidAcquired hemophilia A is a condition in which coagulation factor VIII become inactive due to autoantibody formation. This condition is related to pregnancy, malignancy, and autoimmune disease with skin disorder. In this case report, a 66 years woman with a post procedural bleeding with skin disorder. Later on, patient diagnosed with acquired hemophilia A with a factor VIII inhibitors related to bullous pemphigoid. Keywords : Acquired hemophilia A, bullous pemphigoid, diagnosis, treatment

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