Fujimura Taku, Okuyama Ryuhei, Nakagawa Satoshi, Terui Tadashi, Aiba Setsuya
Department of Dermatology, Tohoku University Graduate School of Medicine, Sendai, Japan.
Dermatology. 2006;212(4):366-9. doi: 10.1159/000092288.
We describe an 81-year-old Japanese patient with erythroderma overlapping with widespread and symmetrical deposits of mucin in the upper dermis. Clinically, the mucinous lesions on the nape and upper trunk were localized papular mucinosis. Histologically, there was a perivascular infiltrate of lymphohistiocytic cells mingled with plasma cells in the upper dermis but no sclerosis. Immunohistochemical staining revealed that more than 90% of these infiltrating plasma cells produced immunoglobulin lambda-chain. Both the erythroderma and generalized mucinosis responded to topical steroid and PUVA therapy. To the best of our knowledge, this is the first case of erythroderma accompanied by generalized mucinosis.
我们描述了一名81岁的日本患者，患有红皮病，同时在上层真皮中有广泛且对称的粘蛋白沉积。临床上，颈部和上躯干的粘液性病变为局限性丘疹性粘蛋白病。组织学上，上层真皮中有淋巴细胞和组织细胞的血管周围浸润，并伴有浆细胞，但无硬化。免疫组化染色显示，这些浸润的浆细胞中超过90%产生免疫球蛋白λ链。红皮病和全身性粘蛋白病对局部类固醇和PUVA治疗均有反应。据我们所知，这是首例伴有全身性粘蛋白病的红皮病病例。
