Acquired haemophilia heralded by bleeding into the oral mucosa in a patient with bullous pemphigoid, rheumatoid arthritis, and vitiligo

Patel, Rakesh; Harman, K. E.; Nichols, C; Burd, Robert M.; Pavord, Sue

doi:10.1136/pgmj.2005.036483

Cited by 24 publications

(20 citation statements)

References 6 publications

(5 reference statements)

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“…Presenting symptoms of BP associated AH include hematomas, ecchymosis, gastrointestinal bleeding and oral mucosal bleeding. This was compatible with the history of lip hematoma in our patient [9][10][11][12][13][14][15][16][17][18][19].…”

Section: Discussionsupporting

confidence: 89%

Suprachoroidal Hemorrhage with Delayed Rebleeding as the First Presentation of Bullous Pemphigoid Associated Acquired Hemophilia

Mak¹,

Shum²,

Choy³

et al. 2016

J Clin Exp Ophthalmol

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We report a case of phacoemulsification conversion complicated by suprachoroidal haemorrhage (SCH) and delayed rebleeding at day 5 postoperation, as the first presentation of bullous pemphigoid associated acquired hemophilia. This association has been reported sparingly in the past, and almost exclusively only in hematology literature. To the best of our knowledge, this is the first case of delayed SCH rebleed presenting in a patient with previously undiagnosed Acquired Hemophilia reported. Although exceedingly rare, acquired hemophilia leads to drastic complications and has a high mortality rate. Advice to allow early recognition and prevention of complications is summarized.

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Section: Discussionsupporting

confidence: 89%

Suprachoroidal Hemorrhage with Delayed Rebleeding as the First Presentation of Bullous Pemphigoid Associated Acquired Hemophilia

Mak¹,

Shum²,

Choy³

et al. 2016

J Clin Exp Ophthalmol

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“…This should be revealed through the course of a detailed medical history and examination. Reported associations include systemic lupus erythematosus, rheumatoid arthritis, multiple sclerosis, Sjögren syndrome and Myasthenia gravis which was of relevance in this case. The dental clinician in particular should make careful note of oral and dermatological manifestations of autoimmunity including psoriasis, epidemolysis bullosa, pemphigus and bullous pemphigoid …”

Section: Discussionmentioning

confidence: 78%

“…The dental clinician in particular should make careful note of oral and dermatological manifestations of autoimmunity including psoriasis, epidemolysis bullosa, pemphigus and bullous pemphigoid. 19 Bleeding can be iatrogenic or induced by minor trauma. This is of particular relevance following invasive oral surgery procedures that produce intra-oral wounds with bone exposure in highly vascular areas.…”

Section: Discussionmentioning

confidence: 99%

Acquired haemophilia A: a clinical case

2012

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Background Acquired haemophilia A (AHA) is a rare medical emergency that may be characterised by severe and life‐threatening haemorrhage. Aims This case report highlights the need for a better awareness of this disorder in terms of diagnosis and urgent management. Materials and Methods This is a clinical presentation of a 77‐year‐old Caucasian man who presented to Accident and Emergency with profuse bleeding from his tongue, secondary to mechanical trauma. Local haemostatic measures were only temporarily effective as he returned on a further five occasions over a 3‐week period with recurrent bleeding from the same site. A coagulation screen revealed prolongation of the activated partial thromboplastin time that did not correct on a 50/50 mixing study with normal plasma. Subsequent analysis of intrinsic coagulation factor activity levels revealed severe factor VIII (FVIII) deficiency. A high‐titre FVIII inhibitor was identified in his plasma and the diagnosis of AHA was made. The patient was promptly transferred to the regional haemophilia comprehensive care centre for specialist management. Discussion AHA should be suspected in a patient with no personal or family history of a bleeding disorder who presents with soft tissue haemorrhage unresponsive to local measures. Conclusion This condition is particularly relevant to dental clinicians as patients may present with oral clinical signs of disease. In addition, affected individuals are also at risk of severe haemorrhage following accidental trauma or invasive oral surgery procedures.

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“…2,19,[25][26][27][28] It is also reported that 2-8.3% of AH cases are associated with skin disorders: these include psoriasis vulgaris, bullous pemphigoid, exfoliative dermatitis, pemphigus vulgaris, erythema multiforme, erythema annulare centrifugum, scleroderma, malignant melanoma and nonspecific dermatoses. 2,6,7,19,25,26,29,30 Reported cases of autoimmune bullous dermatoses associated with AH include pemphigus vulgaris, [31][32][33] pemphigus erythematosus, 34 bullous pemphigoid, [35][36][37][38][39][40][41][42][43][44] epidermolysis bullosa acquisita [45][46][47] and anti-laminin 5 pemphigoid. 48 However, to the best of our knowledge, there have not been any previous reports of LABD associated with AH, so the present report may be the first case of LABD associated with AH.…”

Section: Discussionmentioning

confidence: 99%

Case of linear immunoglobulin A bullous dermatosis associated with acquired hemophilia

Arakaki

Yamamoto

Awazawa

et al. 2008

The Journal of Dermatology

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Linear immunoglobulin (Ig)A bullous dermatosis is a rare autoimmune subepidermal bullous dermatosis caused by circulating IgA autoantibodies directed against the antigens at the basement membrane zone. Most linear IgA bullous dermatosis cases are idiopathic, but some are associated with the use of certain drugs, infections, lymphoproliferative disorders, internal malignancies, autoimmune disorders, collagen diseases or, very rarely, other skin diseases, including autoimmune bullous diseases. Acquired hemophilia is also rare; it is a coagulation disease caused by anti-factor VIII IgG antibodies. Acquired hemophilia has been reported to be associated with malignant tumors, pregnancy or postpartum, drug reactions, collagen diseases such as rheumatoid arthritis, autoimmune disorders, and skin diseases such as psoriasis and pemphigus. We report a case of hemophilia acquired during the course of linear IgA bullous dermatosis and review reported cases of autoimmune bullous dermatoses associated with acquired hemophilia.

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Acquired haemophilia heralded by bleeding into the oral mucosa in a patient with bullous pemphigoid, rheumatoid arthritis, and vitiligo

Cited by 24 publications

References 6 publications

Suprachoroidal Hemorrhage with Delayed Rebleeding as the First Presentation of Bullous Pemphigoid Associated Acquired Hemophilia

Suprachoroidal Hemorrhage with Delayed Rebleeding as the First Presentation of Bullous Pemphigoid Associated Acquired Hemophilia

Acquired haemophilia A: a clinical case

Case of linear immunoglobulin A bullous dermatosis associated with acquired hemophilia

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