Division of Hematology/Oncology, Brookdale University Hospital & Medical Center, Brooklyn, NY, 11212, USA.
Exp Hematol Oncol. 2012 Aug 13;1(1):19. doi: 10.1186/2162-3619-1-19.
Two major types of amyloidosis are primary amyloidosis or amyloid light chain amyloidosis and secondary amyloidosis. Although amyloidosis involves a variety of organ systems including skin, the occurrence of bullous skin lesions is rare. Little is known about the mechanism of blister formation. These blisters are often hemorrhagic and typically occur in the oral mucosa. Only a few case reports have described skin involvement in systemic amyloidosis. The manifestation of bullous lesions on the breast in association with primary amyloidosis has not been previously reported. Therefore, we report a case of cutaneous hemorrhagic bullous of the breast secondary to primary systemic amyloidosis, which may be important for medical oncologists to be aware of this uncommon presentation of plasma cell dysrasias. Furthermore, this case only partially responded to the commonly used multiple myeloma-type regimen, the skin lesions responded completely to a five-drug combination chemotherapy regimen, utilizing immunomodulators, liposomal doxorubicin, cyclophosphamide, bortezomib, and dexamethasone, suggesting that a more aggressive modality of chemotherapy may be necessary to treat such cases.
两种主要类型的淀粉样变性是原发性淀粉样变性或轻链淀粉样变性和继发性淀粉样变性。尽管淀粉样变性涉及多种包括皮肤在内的器官系统,但水疱性皮肤病变的发生较为罕见。对于水疱形成的机制知之甚少。这些水疱通常是出血性的,通常发生在口腔黏膜。仅有少数病例报告描述了系统性淀粉样变性的皮肤受累。原发性淀粉样变性相关的乳房大疱性病变的表现以前没有报道过。因此,我们报告了一例继发于原发性系统性淀粉样变性的乳房皮肤出血性大疱,这可能对肿瘤内科医生了解浆细胞疾病的这种不常见表现很重要。此外,该病例对常用的多发性骨髓瘤样方案仅部分有反应,皮肤病变对包括免疫调节剂、脂质体多柔比星、环磷酰胺、硼替佐米和地塞米松的五药联合化疗方案完全有反应,这表明可能需要更积极的化疗方式来治疗此类病例。