Abstract
Epidermolysis bullosa acquisita (EBA) is a rare chronic subepidermal bullous autoimmune disease. The occurrence of acquired hemophilia A (AHA) is low and so the coexistence of EBA and AHA is extremely rare. We herein described a case of EBA coexisting with AHA and a case of EBA coexisting with AHA and hepatitis B. These EBA may be related to the pathogenesis of AHA. In this study, we analyzed the clinical features in the two Chinese cases of EBA coexisting with AHA, and found esophageal hemorrhage and hematemesis were the main symptoms of both patients. Cyclosporin, prednisone and lamivudine effectively control EBA with AHA and hepatitis B. The dose of cyclosporin should be more than 4 mg/kg per day and the period of treatment should be longer than 5 months to reduce the risk of EBA co-occurring with AHA.
Keywords:
acquired hemophilia A; cyclosporin; epidermolysis bullosa acquisita; hepatitis B; lamivudine.
© 2016 Japanese Dermatological Association.
MeSH terms
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Adult
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Autoantibodies / analysis
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Autoimmune Diseases / blood
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Autoimmune Diseases / complications*
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Autoimmune Diseases / drug therapy
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Autoimmune Diseases / pathology
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Biopsy
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China
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Collagen Type VII / immunology
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Cyclosporine / administration & dosage
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Cyclosporine / therapeutic use
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Epidermolysis Bullosa Acquisita / blood
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Epidermolysis Bullosa Acquisita / complications*
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Epidermolysis Bullosa Acquisita / drug therapy
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Epidermolysis Bullosa Acquisita / pathology
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Esophageal Diseases / etiology*
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Factor VIII / immunology
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Gastrointestinal Hemorrhage / etiology*
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Glucocorticoids / administration & dosage
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Glucocorticoids / therapeutic use
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Hematemesis / etiology*
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Hemophilia A / blood
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Hemophilia A / complications*
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Hemophilia A / drug therapy
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Hepatitis B / blood
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Hepatitis B / complications*
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Hepatitis B / drug therapy
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Humans
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Immunosuppressive Agents / administration & dosage
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Immunosuppressive Agents / therapeutic use
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Lamivudine / administration & dosage
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Lamivudine / therapeutic use
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Male
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Middle Aged
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Prednisone / administration & dosage
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Prednisone / therapeutic use
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Reverse Transcriptase Inhibitors / administration & dosage
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Reverse Transcriptase Inhibitors / therapeutic use
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Skin / pathology
Substances
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Autoantibodies
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Collagen Type VII
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Glucocorticoids
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Immunosuppressive Agents
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Reverse Transcriptase Inhibitors
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Lamivudine
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Cyclosporine
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Factor VIII
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Prednisone