Chang Hyun Kyu, Kim You Chan, Kwon Bum Sun
Division of Rheumatology, Department of Internal Medicine, Dankook University Hospital, Cheonan, Korea.
J Korean Med Sci. 2004 Jun;19(3):481-3. doi: 10.3346/jkms.2004.19.3.481.
Scleredema is a rare cutaneous mucinosis characterized by chronic diffuse induration of the skin, and it is occasionally associated with a monoclonal gammopathy (MG). Ankylosing spondylitis (AS) is noted to be another, chronic systemic inflammatory disorder of the axial skeleton that may accompany the MG. However, patients with scleredema and AS accompanied with a MG have not been reported in the literature. We here report a 40-yr-old man with scleredema and advanced AS accompanied with a MG of IgA-kappa protein. Widespread, long-standing scleredema has been developed over 10 yrs after the initial manifestation of AS. It is uncertain whether the coexistence of scleredema and AS is more than coincidental.
硬化性黏液水肿是一种罕见的皮肤黏蛋白病,其特征为皮肤慢性弥漫性硬结,偶尔与单克隆丙种球蛋白病(MG)相关。强直性脊柱炎(AS)是另一种可伴随MG的慢性系统性中轴骨骼炎性疾病。然而,文献中尚未报道过伴有MG的硬化性黏液水肿和AS患者。我们在此报告一名40岁男性,患有硬化性黏液水肿和晚期AS,并伴有IgA-κ蛋白的MG。广泛性、长期存在的硬化性黏液水肿在AS最初表现出现10多年后发生。硬化性黏液水肿和AS并存是否只是巧合尚不确定。