Recalcitrant facial pemphigus vulgaris: correlation of skin lesions with the ratio of antidesmoglein antibodies 1 and 3

• Published in: Clinical and experimental dermatology Vol. 36; no. 3; pp. 284 - 287
• Main Authors: Fujii, M., Honma, M., Iinuma, S., Kaneta, K., Komatsu, S., Sato, K., Takahashi, H., Ishida-Yamamoto, A., Iizuka, H.
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Publishing Ltd 01-04-2011; Wiley-Blackwell; Oxford University Press
• Subjects: Autoantibodies - blood; Biological and medical sciences; Bullous diseases of the skin; Dermatology; Desmoglein 1 - immunology; Desmoglein 3 - immunology; Facial Dermatoses - immunology; Facial Dermatoses - pathology; Female; Geriatrics; Humans; Medical sciences; Medical treatment; Middle Aged; Pemphigus - immunology; Pemphigus - pathology; Bullous dermatosis; Immunopathology; Skin disease; Antibody; Dermatology; Autoimmune disease; Pemphigus vulgaris; Face
• ISSN: 0307-6938; 1365-2230
• DOI: 10.1111/j.1365-2230.2010.03920.x

Summary
Pemphigus vulgaris (PV) is an autoimmune bullous disease characterized by autoantibodies against desmogleins. We report a case of recalcitrant PV, which progressed from the mucosal to the mucocutaneous type, with a corresponding increase in anti‐desmoglein (Dsg)1 and decrease in anti‐Dsg3 antibody titres. Thus, the clinical features seemed to correlate with the ratio of anti‐Dsg1 and 3. The patient also had anti‐Dsg4 antibodies, which might be related to the nonscarring diffuse hair loss and marked facial involvement she also had. The patient did not respond to treatment with systemic steroid, ciclosporin, azathioprine, cyclophosphamide or double filtration plasmapheresis, and eventually died from fulminant thrombotic thrombocytopenic purpura of unknown cause.