[Simultaneous onset of pemphigoid and factor VIII antibody hemophilia].
A 47-year old patient who had been suffering from hypertension and chronic renal failure for many years developed progressive extensive haemorrhagic erosions of the mouth within 3 months and less severe erosions of the genital and nasal mucosa. Additionally, subcutaneous haematomas developed spontaneously. Laboratory investigations demonstrated circulating antibodies against factor VIII while direct and indirect immunofluorescent microscopy showed discrete tissue-bound and circulating IgG reactive with the epidermal basement membrane in a pemphigoid-like fashion. Immunoblot analysis of the patient's serum revealed an "atypical" IgG reactivity against a central portion of the extracellular domain of the BP180 antigen. These findings were unexpected, since the clinical aspect showed striking resemblance to (paraneoplastic) pemphigus. The patient developed life-threatening complications. Eventually, reduction of circulating autoantibodies by a combination of plasmapheresis and subsequent immunosuppressive therapy led to a stable remission of both autoimmune bullous skin disorder and acquired haemophilia.
Maczek
C
C
Dermatologische Universitätsklinik, Universität Erlangen-Nürnberg.
Thoma-Uszynski
S
S
Schuler
G
G
Hertl
M
M
D001323:Autoantibodies / Q000097:blood*; D001324:Autoantigens / Q000276:immunology; D001327:Autoimmune Diseases / Q000150:complications* / Q000276:immunology / Q000473:pathology; D001706:Biopsy; D003937:Diagnosis, Differential; D005169:Factor VIII / Q000276:immunology*; D006467:Hemophilia A / Q000150:complications* / Q000276:immunology / Q000473:pathology; D006474:Hemorrhagic Disorders / Q000150:complications / Q000276:immunology / Q000473:pathology; D006801:Humans; D015151:Immunoblotting; D007074:Immunoglobulin G / Q000097:blood; D007676:Kidney Failure, Chronic / Q000150:complications / Q000276:immunology / Q000473:pathology; D008297:Male; D008856:Microscopy, Fluorescence; D008875:Middle Aged; D009061:Mouth Mucosa / Q000276:immunology / Q000473:pathology; D024041:Non-Fibrillar Collagens; D010391:Pemphigoid, Bullous / Q000150:complications* / Q000276:immunology / Q000473:pathology; D012867:Skin / Q000276:immunology / Q000473:pathology; D000097805:Collagen Type XVII
D001323:Autoantibodies; D001324:Autoantigens; D005169:Factor VIII; D007074:Immunoglobulin G; D024041:Non-Fibrillar Collagens; D000097805:Collagen Type XVII