Section of Dermatology and Department of Dermatopathology, University of Genoa, Genoa, Italy.
J Am Acad Dermatol. 2012 Jul;67(1):128-35. doi: 10.1016/j.jaad.2011.09.008. Epub 2011 Oct 22.
Pseudoxanthoma elasticum (PXE)-like papillary dermal elastolysis (PDE) is a rare acquired elastic tissue disorder. To date, less than 20 cases have been reported.
We report a case series of 17 patients presenting with PXE-like PDE and discuss the clinicopathological correlation.
Seventeen cases of PXE-like PDE were collected prospectively and evaluated for common demographic, clinical, and histopathological features.
All patients were women with a mean age of 61.8 years. The lateral sides and back of neck were the most common sites of involvement (100%), followed by the supraclavicular region (41.2%) and the axilla (35.3%). Systemic involvement was absent in all cases, and in 7 patients the discovery of PXE-like PDE was an incidental finding. The main histopathologic features included complete loss (82.4%) or marked reduction (17.6%) of elastic fibers in the papillary dermis and the presence of melanophages in the same zone (88.2%).
Our results require validation with a larger series.
Our findings help to differentiate PXE-like PDE from similar elastic tissue disorders based on the selective elastic tissue elimination in the papillary dermis and the presence of melanophages in the same zone as a possible consequence of subclinical junctional photodamage. PXE-like PDE is likely underdiagnosed rather than rare, and dermatologists should be aware of its similarity to inherited PXE to spare unnecessary investigations because of the lack of systemic involvement. Clinicopathologic correlation is critical as hematoxylin-eosin staining is nonspecific and elastic tissue stains are necessary to make the correct diagnosis.
假性弹力纤维瘤病（PXE）样乳头真皮弹力纤维松解症（PDE）是一种罕见的获得性弹性组织疾病。迄今为止，报道的病例不足 20 例。
我们报告了一组 17 例 PXE 样 PDE 患者，并讨论了其临床病理相关性。
前瞻性收集了 17 例 PXE 样 PDE 病例，并评估了其常见的人口统计学、临床和组织病理学特征。
所有患者均为女性，平均年龄 61.8 岁。病变最常累及颈侧和背部（100%），其次为锁骨上区（41.2%）和腋窝（35.3%）。所有病例均无系统受累，7 例发现 PXE 样 PDE 为偶然发现。主要的组织病理学特征包括乳头真皮中弹性纤维完全缺失（82.4%）或明显减少（17.6%），以及同一区域存在黑素细胞（88.2%）。
我们的结果需要更大系列的验证。
我们的发现有助于根据乳头真皮中选择性弹性组织消除以及同一区域存在黑素细胞（可能是亚临床交界性光损伤的结果）将 PXE 样 PDE 与类似的弹性组织疾病区分开来。PXE 样 PDE 可能被误诊而非罕见，由于缺乏系统受累，皮肤科医生应该意识到其与遗传性 PXE 的相似性，以免因不必要的检查而误诊。临床病理相关性至关重要，因为苏木精-伊红染色不具有特异性，需要进行弹性组织染色才能做出正确诊断。
