Atypical Presenting Symptoms of Acute Onset Acquired Haemophilia with Eosinophilic Fasciitis
  • Yuto Nakakubo
    Department of Clinical Immunology and Rheumatology, The Tazuke-Kofukai Medical Research Institute, Kitano Hospital, Osaka, Japan
  • Kazuyo Yamamoto
    Department of Hematology, The Tazuke-Kofukai Medical Research Institute, Kitano Hospital, Osaka, Japan
  • Masaaki Fujita
    Department of Clinical Immunology and Rheumatology, Kansai Electric Power Hospital, Osaka, Japan


Acquired haemophilia, eosinophilic fasciitis


Acquired haemophilia is a bleeding disorder caused by antibodies against coagulation factors. Some cases are associated with autoimmune diseases. However, no cases of acquired haemophilia with eosinophilic fasciitis have been previously reported. Herein we describe the case of a patient with eosinophilic fasciitis associated with acquired haemophilia.



  • O’Connor CR. Systemic review of the presentation of coagulation factor VIII inhibitors in rheumatic diseases: a potential cause of life-threatening hemorrhage. Semin Arthritis Rheum 2015;44:695–709.

  • Kruse-Jarres R, Kempton CL, Baudo F, Collins PW, Knoebl P, Leissinger CA, et al. Acquired hemophilia A: updated review of evidence and treatment guidance. Am J Hematol 2017;92:695–705.

  • Green D, Lechner K. A survey of 215 non-hemophilic patients with inhibitors to Factor VIII. Thromb Haemost 1981;45:200–203.

  • Collins PW, Hirsch S, Baglin TP, Dolan G, Hanley J, Makris M, et al. Acquired hemophilia A in the United Kingdom: a 2-year national surveillance study by the United Kingdom Haemophilia Centre Doctors’ Organisation. Blood 2007;109:1870–1877.

  • Tay L, Duncan E, Singhal D, Al-Qunfoidi R, Coghlan D, Jaksic W, et al. Twelve years of experience of acquired hemophilia A: trials and tribulations in South Australia. Semin Thromb Hemost 2009;35:769–777.

  • Knoebl P, Marco P, Baudo F, Collins P, Huth-Kühne A, Nemes L, et al. Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2). J Thromb Haemost 2012;10:622–631.

  • Borg JY, Guillet B, Le Cam-Duchez V, Goudemand J, Lévesque H. Outcome of acquired haemophilia in France: the prospective SACHA (Surveillance des Auto antiCorps au cours de l’Hemophilie Acquise) registry. Haemophilia 2013;19:564–570.

  • Huang SY, Tsay W, Lin SY, Hsu SC, Hung MH, Shen MC. Study of 65 patient with acquired hemophilia A in Taiwan. J Formos Med Assoc 2015;114:321–327.

  • Tiede A, Klamroth R, Scharf RE, Trappe RU, Holstein K, Huth-Kühne A. Prognostic factors for remission of and survival in acquired hemophilia A (AHA): results from GTH-AH 01/2010 study. Blood 2015;125:1091–1097.

  • Ogawa Y, Yanagisawa K, Uchiyama Y, Akashi N, Mieda T, Iizuka H. Clinical characteristics and outcomes of acquired hemophilia A: experience at a single center in Japan. Int J Hematol 2017;106:82–89.

  • Lakhanpal S, Ginsburg WW, Michet CJ, Doyle JA, Moore SB. Eosinophilic fasciitis: clinical spectrum and therapeutic response in 52 cases. Semin Arthritis Rheum 1988;17:221–231.

  • Jinnin M, Yamamoto T, Asano Y, Ishikawa O, Sato S, Takehara K, et al. Diagnostic criteria, severity classification and guidelines of eosinophilic fasciitis. J Dermatol 2018;45:881–890.

  • Lebeaux D, Francès C, Barete S, Wechsler B, Dubourg O, Renoux J, et al. Eosinophilic fasciitis (Shulman disease): new insights into the therapeutic management from a series of 34 patients. Rheumatology (Oxford) 2012;51:557–561.
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    Published: 2020-06-15
    Issue: 2020: Vol 7 No 9 (view)

    How to cite:
    Nakakubo Y, Yamamoto K, Fujita M. Atypical Presenting Symptoms of Acute Onset Acquired Haemophilia with Eosinophilic Fasciitis. EJCRIM 2020;7 doi:10.12890/2020_001722.