Gonzalez-Rodriguez Antonio J, Bella-Navarro Rebeca, Ramon Quiles Dolores, Jorda-Cuevas Esperanza
Hospital Clínico Universitario de Valencia.
Dermatol Online J. 2014 May 16;20(5):22611.
Cutis laxa is a rare entity characterized clinically by redundant skin that gives an appearance of premature aging. The appearance relates to a loss of elasticity because of the destruction of elastic fibers that affects the skin and other organs. It may be associated with inflammatory conditions or diseases, such as plasma cell dyscrasias. We report the case of a 54-year-old man with acquired cutis laxa, which preceded the development of IgG-lambda monoclonal gammopathy with lambda light chain deposits in the kidney. The patient had a fatal outcome owing to severe and rapidly progressive renal failure. We emphasize the importance of recognizing a plasma cell dyscrasia in a patient with cutis laxa, although this association is rare.
皮肤松弛症是一种罕见的病症，其临床特征为皮肤多余，呈现出早衰的外观。这种外观与弹性丧失有关，原因是影响皮肤和其他器官的弹性纤维遭到破坏。它可能与炎症性病症或疾病相关，如浆细胞发育异常。我们报告一例54岁男性获得性皮肤松弛症病例，该病例先于IgG-λ单克隆丙种球蛋白病及肾脏λ轻链沉积的发生。患者因严重且快速进展的肾衰竭而死亡。我们强调，尽管皮肤松弛症患者合并浆细胞发育异常的情况罕见，但认识到这一点很重要。
