TY - JOUR
T1 - Cicatricial pemphigoid with autoantibodies against the laminin 5 gamma 2 subunit
AU - Dainichi, Teruki
AU - Takeshita, Hiromichi
AU - Moroi, Yoichi
AU - Urabe, Kazunori
AU - Yoshida, Mariko
AU - Hisamatsu, Yoshiko
AU - Komai, Ayako
AU - Duan, Hong
AU - Koga, Tetsuya
AU - Hashimoto, Takashi
AU - Furue, Masutaka
PY - 2005/5
Y1 - 2005/5
N2 - 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.
AB - 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.
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M3 - Article
C2 - 15908306
AN - SCOPUS:21144451649
VL - 15
SP - 189
EP - 193
JO - European Journal of Dermatology
JF - European Journal of Dermatology
SN - 1167-1122
IS - 3
ER -