Oesophageal bullous detachment with massive haemorrhage revealing acquired haemophilia A in autoimmune bullous dermatosis

• Published in: Annales de dermatologie et de vénéréologie 2020-03, Vol.147 (3), p.217
• Main Authors: Bonigen, J, Laly, P, Tran-Minh, M-L, Hau, E, Guibal, F, Bagot, M, Petit, A
• Format: Article
• Language: eng ; fre
• ISSN: 0151-9638
• DOI: 10.1016/j.annder.2019.09.598

Acquired haemophilia A (AHA) is a rare coagulopathy caused by the development of factor VIII antibodies. Various aetiologies have been established but a number of cases have been reported in association with autoimmune bullous dermatosis (AIBD). We report a new case of this type of association revealed by oesophageal involvement of AIBD. A male patient was treated for AIBD. Due to the inefficacy of local steroids and the emergence of oral and laryngeal blisters, the patient was treated with systemic steroids. He developed a gastrointestinal haemorrhage complicated by haemorrhagic shock. Endoscopy revealed complete peeling of the oesophagus. Laboratory tests showed lengthening of ACT, reduced factor VIII levels, and the presence of anti-factor VIII antibodies. A diagnosis was made of AHA associated with AIBD. Prolongation of systemic corticosteroids and initiation of rituximab resulted in normalisation of haemostasis. AIBD and AHA frequently develop concomitantly, as was the case with our patient. The haemorrhagic complications were severe. The aim of AHA treatment is to stop acute bleeding and eliminate antibodies, and for this reason rituximab was chosen. Oesophageal bullous detachment is rare in AIBD but, as seen here, it may be responsible for massive haemorrhage, especially in the event of associated AHA. This feature underscores the need for evaluation of haemostasis in the early stages and during relapses for all patients with AIBD.