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Journal of the American Academy of Dermatology
Volume 42, n° 5P1
pages 841-844 (mai 2000)
Doi : 10.1067/mjd.2000.104790
Antiepiligrin cicatricial pemphigoid
 

Roger Chin-Che Hsu, MD a, Zelmira Lazarova, MD b, Han-Ghieh Lee, MD c, Yi-Ching Tung, MS d, Hsin-Su Yu, MD, PhD a
a Department of Dermatology, Kaohsiung Medical College Kaohsiung, Taiwan 
b Dermatology Branch, National Institutes of Health Kaohsiung, Taiwan 
c Armed Forces Tso-Ying Hospital Kaohsiung, Taiwan 
d Department of Microbiology, Kaohsiung Medical College. Kaohsiung, Taiwan 

Abstract

Cicatricial pemphigoid is a chronic subepithelial autoimmune blistering disease of mucous membranes and skin. Recently, a subtype of cicatricial pemphigoid with autoantibodies to epiligrin was identified. We describe a Taiwanese patient who presented with ocular, oral, and cutaneous involvement. Direct immunofluorescence showed IgG and C3 deposition in epidermal basement membrane; indirect immunofluorescence showed circulating IgG autoantibodies reactive with the dermal side of 1 mol/L sodium chloride–split skin. Immunoblotting of laminin 5 isolated from the extracellular matrix of cultured human keratinocytes showed no specific reactivity. In contrast, with immunoprecipitation of the conditioned culture media from biosynthetically radiolabeled human keratinocytes, this patient’s serum clearly reacted with a series of disulfide-linked polypeptides that correspond to laminin 5(⍺3β3γ2) and laminin 6(⍺3β1γ1). This is the first confirmed case of a patient of Chinese ancestry with this disease entity. (J Am Acad Dermatol 2000;42:841-4.)

The full text of this article is available in PDF format.

 Reprint requests: Hsin-Su Yu, MD, PhD, Department of Dermatology, Kaohsiung Medical College Hospital, Shih-Chuan 1st Rd, Kaohsiung 80708 Taiwan.



© 2000  American Academy of Dermatology, Inc. Published by Elsevier Masson SAS@@#104157@@
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