Journal of Dermatology 2015; 42: 394–397
doi: 10.1111/1346-8138.12798
CONCISE COMMUNICATION
Pemphigoid with antibodies to laminin c1, BP180 and BP230, associated with psoriasis vulgaris: Successful disease control with cyclosporin Akiko IMANISHI,1 Chiharu TATEISHI,1 Hisayoshi IMANISHI,1 Junko SOWA-OSAKO,1 Hiroshi KOGA,2 Daisuke TSURUTA,1 Takashi HASHIMOTO2 1
Department of Dermatology, Osaka City University Graduate School of Medicine,Osaka, 2Department of Dermatology, Kurume University School of Medicine, Kurume University Institute of Cutaneous Cell Biology, Fukuoka, Japan
ABSTRACT Both anti-laminin c1 pemphigoid and bullous pemphigoid are autoimmune subepidermal blistering diseases. The former is rare and characterized by autoantibodies to laminin c1, a 200-kDa dermal protein, while the latter is common among the elderly and characterized by autoantibodies to BP180 and BP230, both of which are hemidesmosomal proteins. We experienced a 69-year-old Japanese male patient with blister formation secondary to erythrodermic psoriasis, which was successfully treated with cyclosporin. The histopathology of erythema corresponded with psoriasis and that of a blistering lesion showed infiltration of neutrophils and eosinophils in and around the subepidermal blisters. Patient immunoglobulin G antibodies labeled both the epidermal and dermal sides of 1 mol/L NaCl-split human skin by indirect immunofluorescent microscopy and recognized laminin c1, BP180 and BP230 by immunoblotting. To the best of our knowledge, this is the first report of coexistence of psoriasis and atypical pemphigoid with these three autoantibodies.
Key words:
BP180, cyclosporin, laminin c1, pemphigoid, psoriasis.
INTRODUCTION Anti-laminin c1 pemphigoid is a rare, autoimmune, subepidermal, blistering disease characterized by circulating autoantibodies of the patients which react with laminin c1 subunit, a 200-kDa dermal protein (p200), and 90% of cases of anti-p200 pemphigoid are reported to be anti-laminin c1 pemphigoid.1,2 BP180 and BP230 are target antigens of bullous pemphigoid (BP), the most popular autoimmune subepidermal blistering disease of elderly people in Japan.3 Psoriasis is a chronic inflammatory skin disease characterized by well-circumscribed, erythematous and squamous papules and plaques with silvery scales. In severe case, it may affect the entire body and progress into generalized erythrodermic psoriasis.4 Here, we report the first case of coexistence of erythrodermic psoriasis and pemphigoid with anti-laminin c1, anti-BP180 and antiBP230 antibodies, which was successfully treated with cyclosporin.
CASE REPORT A 69-year-old Japanese man visited our hospital because of pruritic erythemas with a 3-day history of blister formation. He had suffered from psoriasis vulgaris for 16 years and was
treated with topical corticosteroid and vitamin D3. However, he had stopped the ointments 2 months prior. On examination, erythemas with thick scales were seen on the entire body, except for the face (Fig. 1a). In addition, multiple tense blisters and erosions developed on the forearms and nape (Fig. 1b). No mucosal involvement was observed. Laboratory examinations revealed elevated white blood cell count (10 400/lL) with increases in eosinophils (63%), C-reactive protein (2.69 mg/L) and immunoglobulin (Ig)E level (3000 lg/L). Using enzyme-linked immunosorbent assays (ELISA) (MBL, Nagoya, Japan), antibodies to both BP180 (90 index: normal,
doi: 10.1111/1346-8138.12798
CONCISE COMMUNICATION
Pemphigoid with antibodies to laminin c1, BP180 and BP230, associated with psoriasis vulgaris: Successful disease control with cyclosporin Akiko IMANISHI,1 Chiharu TATEISHI,1 Hisayoshi IMANISHI,1 Junko SOWA-OSAKO,1 Hiroshi KOGA,2 Daisuke TSURUTA,1 Takashi HASHIMOTO2 1
Department of Dermatology, Osaka City University Graduate School of Medicine,Osaka, 2Department of Dermatology, Kurume University School of Medicine, Kurume University Institute of Cutaneous Cell Biology, Fukuoka, Japan
ABSTRACT Both anti-laminin c1 pemphigoid and bullous pemphigoid are autoimmune subepidermal blistering diseases. The former is rare and characterized by autoantibodies to laminin c1, a 200-kDa dermal protein, while the latter is common among the elderly and characterized by autoantibodies to BP180 and BP230, both of which are hemidesmosomal proteins. We experienced a 69-year-old Japanese male patient with blister formation secondary to erythrodermic psoriasis, which was successfully treated with cyclosporin. The histopathology of erythema corresponded with psoriasis and that of a blistering lesion showed infiltration of neutrophils and eosinophils in and around the subepidermal blisters. Patient immunoglobulin G antibodies labeled both the epidermal and dermal sides of 1 mol/L NaCl-split human skin by indirect immunofluorescent microscopy and recognized laminin c1, BP180 and BP230 by immunoblotting. To the best of our knowledge, this is the first report of coexistence of psoriasis and atypical pemphigoid with these three autoantibodies.
Key words:
BP180, cyclosporin, laminin c1, pemphigoid, psoriasis.
INTRODUCTION Anti-laminin c1 pemphigoid is a rare, autoimmune, subepidermal, blistering disease characterized by circulating autoantibodies of the patients which react with laminin c1 subunit, a 200-kDa dermal protein (p200), and 90% of cases of anti-p200 pemphigoid are reported to be anti-laminin c1 pemphigoid.1,2 BP180 and BP230 are target antigens of bullous pemphigoid (BP), the most popular autoimmune subepidermal blistering disease of elderly people in Japan.3 Psoriasis is a chronic inflammatory skin disease characterized by well-circumscribed, erythematous and squamous papules and plaques with silvery scales. In severe case, it may affect the entire body and progress into generalized erythrodermic psoriasis.4 Here, we report the first case of coexistence of erythrodermic psoriasis and pemphigoid with anti-laminin c1, anti-BP180 and antiBP230 antibodies, which was successfully treated with cyclosporin.
CASE REPORT A 69-year-old Japanese man visited our hospital because of pruritic erythemas with a 3-day history of blister formation. He had suffered from psoriasis vulgaris for 16 years and was
treated with topical corticosteroid and vitamin D3. However, he had stopped the ointments 2 months prior. On examination, erythemas with thick scales were seen on the entire body, except for the face (Fig. 1a). In addition, multiple tense blisters and erosions developed on the forearms and nape (Fig. 1b). No mucosal involvement was observed. Laboratory examinations revealed elevated white blood cell count (10 400/lL) with increases in eosinophils (63%), C-reactive protein (2.69 mg/L) and immunoglobulin (Ig)E level (3000 lg/L). Using enzyme-linked immunosorbent assays (ELISA) (MBL, Nagoya, Japan), antibodies to both BP180 (90 index: normal,
