Background

This is a rare blistering disease. It is an autoimmune subepidermal blistering disease characterized by an autoantibody directed against a 450kd epidermal antigen. It is a member of the plectin family.

OUTLINE

Epidemiology
Disease Associations
Pathogenesis
Laboratory/Radiologic/ Other Diagnostic Testing
Gross Appearance and Clinical Variants
Histopathological Features and Variants
Special Stains/ Immunohistochemistry/ Electron Microscopy
Differential Diagnosis
Prognosis
Treatment
Commonly Used Terms
Internet Links

PATHOGENESIS	CHARACTERIZATION
Autoantibody directed against 450 kd protein

 

CLINICAL OR GROSS VARIANT

CHARACTERIZATION

A case of subepidermal blister disease associated with autoantibody against 450 kD protein. Fujiwara S, Shinkai H, Takayasu S, Owaribe K, Tsukita S, Kageshita T. Department of Dermatology, Medical College of Oita, Japan.

J Dermatol 1992 Oct;19(10):610-3 Abstract quote We report a case of subepidermal blister disease with eruptions distributed on the trunk and extremities. The blisters regressed after administration of corticosteroid and cyclophosphamide. An immunoblot analysis revealed that the patient's serum reacted exclusively with a 450-kD epidermal polypeptide. It did not react with a 230-kD or a 180-kD bullous pemphigoid antigen. The immunofluorescence study showed the patient's serum reacted with the basement membrane zone of human skin.

J Invest Dermatol 1996;106:1125-1130
Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.

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Last Updated June 15, 2005

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