Chang Shyue-Luen, Lai Ping-Chin, Cheng Chien-Jui, Yang Li-Cheng, Hong Hong-Shang
Department of Dermatology, Chang-Gung Memorial Hospital, No. 199 Tunghaw N. Road, Taipei, Taiwan.
Amyloid. 2007 Jun;14(2):153-6. doi: 10.1080/13506120701259762.
We report a 78-year-old woman on hemodialysis who presented with refractory multiple pruritic vesicles and bullae on her trunk and extremities for 2 months. Histopathologic examination of skin biopsy specimen showed subepidermal bullae with many amyloid deposits in the papillary dermis. No evidence of systemic amyloidosis could be found on physical examination. While the initial clinical diagnosis was bullous pemphigoid, the histopathology and direct immunofluorescence result favored hemodialysis-associated amyloidosis. However, immunochemical study for beta(2)-microglobulin was negative. Further hematologic and immunologic work-up revealed the presence of multiple myeloma and that the deposit was AL amyloid. This is the first case of bullous amyloidosis in a hemodialysis patient and should remind dermatologists that bullous amyloidosis should be considered in addition to the usual presentation of porphyria cutanea tarda and pseudoporphyria for bullous dermatosis in the hemodialysis patient. We also suggest that hemodialysis-associated amyloidosis should not be taken for granted in the hemodialysis patient with cutaneous amyloidosis without systemic signs and symptoms. Further testing for other types of amyloid should be performed.
我们报告了一名78岁接受血液透析的女性,她躯干和四肢出现难治性多发性瘙痒性水疱和大疱2个月。皮肤活检标本的组织病理学检查显示表皮下水疱,乳头真皮层有许多淀粉样沉积物。体格检查未发现系统性淀粉样变的证据。虽然最初的临床诊断为大疱性类天疱疮,但组织病理学和直接免疫荧光结果支持血液透析相关淀粉样变。然而,β2微球蛋白的免疫化学研究为阴性。进一步的血液学和免疫学检查发现存在多发性骨髓瘤,沉积物为AL淀粉样蛋白。这是血液透析患者中首例大疱性淀粉样变病例,应提醒皮肤科医生,对于血液透析患者的大疱性皮肤病,除了迟发性皮肤卟啉病和假性卟啉病的常见表现外,还应考虑大疱性淀粉样变。我们还建议,对于没有全身体征和症状的皮肤淀粉样变血液透析患者,不应想当然地认为是血液透析相关淀粉样变。应进行其他类型淀粉样变的进一步检测。