Frémont G, Kérob D, Prost-Squarcioni C, Lièvre N, Rivet J, Tancrède E, Servant J-M, Fermand J-P, Morel P, Lebbé C
Service de Dermatologie, Hôpital Saint Louis, Paris.
Ann Dermatol Venereol. 2007 Jun-Jul;134(6-7):548-51. doi: 10.1016/s0151-9638(07)89266-0.
Cutis laxa is a rare disorder characterized by loss of elastic tissue. Several organs are often involved such as the skin, lungs, heart, digestive system or genitourinary tract. It may be inherited or acquired, generalized or localized. Its pathogenesis is unclear. Association of acquired cutis laxa with myeloma or plasma cell dyscrasia is very rare. We report a case of acquired cutis laxa associated with a myeloma.
A 59 year-old woman was admitted for skin hyperlaxity present for a number of years. Light microscopic examination of a skin sample revealed fragmented elastic fibers. Electron microscopic examination of the elastic network demonstrated numerous large vacuolated cells with the appearance of macrophages around abnormal elastic and collagen fibers of the reticular dermis. In addition, a stage-1 IgG lambda myeloma was detected. The patient was treated by thalidomide for one year. After this treatment, electron microscopy examination did not reveal any large vacuolated cells in the dermis, and elastic and collagen fibers were not modified and skin laxity seemed to be stabilized.
Acquired cutis laxa may be associated with many systemic diseases or can appear after inflammatory skin diseases. Seven cases of generalized cutis laxa associated with myeloma and four cases associated with plasma cell dyscrasia have been reported in the literature. In our case, as in 2 previously described cases, large vacuolated cells resembling macrophages were seen in the dermis. They were thought to play a role in cutis laxa.
皮肤松弛症是一种罕见的疾病，其特征为弹性组织丧失。常累及多个器官，如皮肤、肺、心脏、消化系统或泌尿生殖道。它可以是遗传性的或后天获得性的，全身性的或局限性的。其发病机制尚不清楚。后天获得性皮肤松弛症与骨髓瘤或浆细胞发育异常相关的情况非常罕见。我们报告一例后天获得性皮肤松弛症合并骨髓瘤的病例。
一名59岁女性因皮肤松弛多年入院。皮肤样本的光镜检查显示弹性纤维断裂。弹性网络的电镜检查发现，在网状真皮的异常弹性纤维和胶原纤维周围有许多外观类似巨噬细胞的大空泡细胞。此外，检测到一期IgG λ骨髓瘤。患者接受沙利度胺治疗一年。治疗后，电镜检查未在真皮中发现任何大空泡细胞，弹性纤维和胶原纤维未发生改变，皮肤松弛似乎得到稳定。
后天获得性皮肤松弛症可能与许多全身性疾病相关，或可在炎性皮肤病后出现。文献报道了7例与骨髓瘤相关的全身性皮肤松弛症病例以及4例与浆细胞发育异常相关的病例。在我们的病例中，如同之前描述的2例病例一样，在真皮中可见类似巨噬细胞的大空泡细胞。它们被认为在皮肤松弛症中起作用。
