Clinical and immunoserological features of 7 cases of anti-p200 pemphigoid
- VernacularTitle:抗p200类天疱疮7例临床及免疫血清学特征分析
- Author:
Yuan WANG
1
;
Zhiliang LI
;
Suo LI
;
Ke JING
;
Chao SUN
;
Guirong LIANG
;
Hanmei ZHANG
;
Suying FENG
Author Information
- Keywords: Pemphigoid, bullous; Skin manifestations; Fluorescent antibody technique, indirect; Blotting, Western; Therapy; Anti-p200 pemphigoid; Laminin γ1, C-terminus
- From: Chinese Journal of Dermatology 2022;55(7):557-561
- CountryChina
- Language:Chinese
- Abstract: Objective:To analyze clinical and immunoserological features of patients with anti-p200 pemphigoid.Methods:Clinical data were collected from patients with confirmed anti-p200 pemphigoid in Hospital of Dermatology, Chinese Academy of Medical Sciences from January 2015 to October 2021, and their clinical and immunoserological characteristics were retrospectively analyzed.Results:Seven patients with anti-p200 pemphigoid were included. Indirect immunofluorescence on salt-split skin (IIF-SSS) showed that serum IgG antibodies of the 7 patients were located in the dermis of the salt-split skin, and Western blot analysis with dermal extracts as substrates revealed a protein band with a relative molecular mass of 200 000. Four patients presented with classic bullous pemphigoid-like skin lesions, 2 initially presented with eczematous lesions, and 1 presented with linear IgA bullous dermatosis-like skin lesions. Circulating IgG antibodies could recognize the recombinant laminin γ1 C-terminal region in 6 cases. Four patients received different doses of systemic glucocorticoids, 1 of whom was resistant to high-dose systemic glucocorticoids (equivalent to 1.4 mg·kg -1·d -1 prednisone) ; 2 responded well to minocycline and dapsone; 1 was lost to follow-up. Four patients achieved complete remission and discontinued the treatment at a mean follow-up of 22.5 months; 2 received complete remissiona on minimal therapy at a mean follow-up of 8 months. Conclusion:Patients with anti-p200 pemphigoid presented with heterogeneous clinical manifestations, and the recombinant C-terminal fragment of laminin γ1 can serve as a reliable antigen substrate for the detection of autoantibodies in patients with anti-p200 pemphigoid; some patients can eventually achieve complete remission off treatment.
