[大疱性淀粉样变揭示轻链λ型骨髓瘤]
[Bullous amyloïdis revealing a light chains lambda myeloma].
作者信息
Reguiaï Z, Aïnine K, Rémy-Leroux V, Perceau G, Derancourt C, Bernard P
机构信息
Service de Dermatologie, Hôpital Robert-Debré, CHU de Reims, 51092 Reims, France.
出版信息
Rev Med Interne. 2006 Sep;27(9):694-8. doi: 10.1016/j.revmed.2006.05.016. Epub 2006 Jun 16.
INTRODUCTION
Although rare, skin lesions are regularly reported in patients with systemic amyloidosis. The existence of bullous skin lesions however is very rare; only thirty cases have been previously reported. We report a new case of bullous amyloidosis revealing a light chains lambda myeloma, and underline the usual characteristics of this type of systemic amyloidosis.
EXEGESIS
An 85-year-old man was hospitalised for a bullous eruption associated with a general asthenia. Bullous amyloidosis revealing a light chains lambda myeloma was diagnosed and confirmed by histopathological examination of a skin biopsy specimen. The patient died of a severe congestive heart failure, 15 days later, due to cardiac involvement of the amyloidosis.
CONCLUSION
Bullous amyloidosis lesions can be an early manifestation of occult dysglobulinemia. Early diagnosis would allow rapid treatment, before onset of systemic amyloidosis, which is often lethal.
引言
尽管皮肤病变在系统性淀粉样变性患者中并不常见，但仍时有报道。然而，大疱性皮肤病变的存在极为罕见，此前仅有30例相关报道。我们报告了一例新的大疱性淀粉样变性病例，该病例揭示了λ轻链骨髓瘤，并强调了此类系统性淀粉样变性的常见特征。
注释
一名85岁男性因大疱性皮疹伴全身乏力入院。通过皮肤活检标本的组织病理学检查确诊为大疱性淀粉样变性伴λ轻链骨髓瘤。15天后，患者因淀粉样变性累及心脏，死于严重的充血性心力衰竭。
结论
大疱性淀粉样变性病变可能是隐匿性球蛋白异常血症的早期表现。早期诊断有助于在系统性淀粉样变性（通常致命）发病前进行快速治疗。
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