Étienne M, Denizon N, Maillard H
Service de dermatologie, centre hospitalier du Mans, 194, avenue Rubillard, 72037 Le Mans cedex 9, France.
Service d'onco-hématologie, centre hospitalier du Mans, 194, avenue Rubillard, 72037 Le Mans cedex 9, France.
Rev Med Interne. 2015 May;36(5):356-8. doi: 10.1016/j.revmed.2014.02.002. Epub 2014 Mar 4.
Mucocutaneous involvement in systemic amyloidosis occurs in 29 to 40%. Purpura and macroglossia are the most frequently signs. Nail involvement is rarely observed. We reported a case of systemic amyloidosis AL revealed by nail abnormalities.
A 76-year-old-man consulted for nail lesions lasting for several months with subungual hyperkeratosis, distal onycholysis and subungual splinter hemorrhages. The remainder of the dermatological examination highlighted a faint purpuric erythema of eyelid that was overlooked. Further diagnostic work-up confirmed the diagnosis of systemic amyloidosis associated with multiple myeloma lambda light chains.
Nail abnormalities in systemic amyloidosis AL are rarely described. They can take many forms and can sometimes be the only cutaneous manifestation of systemic amyloidosis. Skin biopsy of the nail matrix can confirm the diagnosis with a potential risk of sequela onychodystrophy nail. Labial salivary gland biopsy seems to be the most sensitive method for diagnosing systemic amyloidosis.
系统性淀粉样变性中皮肤黏膜受累发生率为29%至40%。紫癜和巨舌是最常见的体征。指甲受累很少见。我们报告了一例以指甲异常为首发表现的AL型系统性淀粉样变性病例。
一名76岁男性因持续数月的指甲病变就诊，表现为甲下角化过度、远端甲剥离和甲下裂片形出血。皮肤科检查的其他发现突出了眼睑轻微的紫癜性红斑，但被忽视了。进一步的诊断检查确诊为与多发性骨髓瘤λ轻链相关的系统性淀粉样变性。
AL型系统性淀粉样变性中的指甲异常很少被描述。它们可以有多种形式，有时可能是系统性淀粉样变性唯一的皮肤表现。甲母质皮肤活检可以确诊，但有导致甲营养不良后遗症的潜在风险。唇唾液腺活检似乎是诊断系统性淀粉样变性最敏感的方法。
