Silveira Laura, Torres Isabelle, Salvino Marco A, Follador Ivonise, Bittencourt Achiléa L
Department of Internal Medicine, Federal University of Bahia, Salvador, Brazil.
Am J Dermatopathol. 2013 Jun;35(4):e67-71. doi: 10.1097/DAD.0b013e31827bceaf.
Acquired cutis laxa (ACL) is a rare connective tissue disorder that affects the skin elastic fibers, resulting in the loss of elasticity. In 50% of cases, this condition is associated with other diseases, particularly plasma-cell dyscrasias. This report describes a case of ACL with unusual clinical and histopathological characteristics. A 29-year-old man presented with diffuse erythematous plaques that had first appeared 5 months previously. Examination revealed multiple flaccid erythematous plaques on his trunk, neck, and skinfolds. Immunophenotyping of bone marrow aspirate revealed 7% of monoclonal plasma cells with lambda light chain expression. Skin biopsy histology revealed foci of interstitial granulomatous reaction. Weigert stain showed a loss of elastic fibers in the dermis, areas with thickened fibers and elastophagocytosis. Immunohistochemistry was positive for CD68. The cutaneous findings enabled an early diagnosis of IgG lambda monoclonal gammopathy to be made. Microscopic examination revealed an interstitial granulomatous reaction and severe alterations in the elastic fibers that varied in intensity in the different biopsies. Curiously, little has been mentioned in the literature regarding the presence of an interstitial granulomatous reaction in ACL. It is our belief that this reaction is secondary to the degenerative process of the elastic fibers.
获得性皮肤松弛症（ACL）是一种罕见的结缔组织疾病，会影响皮肤弹性纤维，导致弹性丧失。在50%的病例中，这种情况与其他疾病有关，特别是浆细胞发育异常。本报告描述了一例具有不寻常临床和组织病理学特征的ACL病例。一名29岁男性出现弥漫性红斑斑块，最初于5个月前出现。检查发现其躯干、颈部和皮肤褶皱处有多个松弛的红斑斑块。骨髓穿刺免疫表型分析显示7%的单克隆浆细胞表达λ轻链。皮肤活检组织学显示间质肉芽肿反应灶。魏格特染色显示真皮中弹性纤维缺失、纤维增厚区域及弹性纤维吞噬现象。免疫组化CD68呈阳性。皮肤表现有助于早期诊断IgG λ单克隆丙种球蛋白病。显微镜检查显示间质肉芽肿反应以及弹性纤维的严重改变，不同活检组织中的改变程度有所不同。奇怪的是，文献中很少提及ACL中存在间质肉芽肿反应。我们认为这种反应是弹性纤维退变过程的继发表现。
