Cicatricial pemphigoid with autoantibodies against the laminin 5 gamma 2 subunit

Teruki Dainichi, Hiromichi Takeshita, Yoichi Moroi, Kazunori Urabe, Mariko Yoshida, Yoshiko Hisamatsu, Ayako Komai, Hong Duan, Tetsuya Koga, Takashi Hashimoto, Masutaka Furue

Research output: Contribution to journalArticlepeer-review

18 Citations (Scopus)

Abstract

Cicatricial pemphigoid (CP) is an autoimmune bullous disease accompanied by mucosal lesions. The majority of patients with CP have autoantibodies against BP180. Anti-laminin 5 (epiligrin) CP is relatively rare. It is known that, in most cases, circulating autoantibodies against laminin 5 in these patients recognize the α3 and/or β3 subunits of this molecule. Here we report a case of anti-laminin 5 CP, which showed IgG autoantibodies against the γ2 subunit of laminin 5 alone. A 50-year-old woman suffered from skin blistering on the trunk and extremities and severe mucosal lesions in the eyes, oral cavities and laryngopharynx. Despite potent systemic steroids, the mucosal lesions and some parts of the skin lesions persisted. Salazosulfapyridine was of value in controlling the laryngopharyngeal lesions and persistent cutaneous blistering, and cyclophosphamide had definite effects especially on ocular lesions. Anti-laminin 5 autoantibodies became undetectable in serum from the patient after the disease was controlled.

Original languageEnglish
Pages (from-to)189-193
Number of pages5
JournalEuropean Journal of Dermatology
Volume15
Issue number3
Publication statusPublished - May 2005

All Science Journal Classification (ASJC) codes

  • Dermatology

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