Grundmann J U, Bonnekoh B, Gollnick H
Department of Dermatology and Venereology, Otto-von-Guericke University, Leipziger Stasse 44, D-39120 Magdeburg, Germany.
Eur J Dermatol. 2000 Mar;10(2):139-42.
In an 86-year-old woman with a multiple myeloma of the IgG lambda subtype a coinciding systemic amyloidosis manifested as a macroglossia, diffuse alopecia and generalized cutaneous involvement. The skin was affected by milium-like papules, petechial haemorrhages and an increased tissue fragility with subsequent blister formation. The typical histology and immunohistology pattern revealed large intradermal amyloid masses, reacting positively with anti-amyloid A antibodies, which surrounded cuff-like dilatated blood capillaries. The abundance of these amyloid deposits led to significant deflexibilization and fragility of the capillaries and the dermal matrix eventually resulting in the haemorrhagic-bullous eruptions. The peculiar feature of the present case is the intensity of bullous-haemorrhagic skin damage due to amyloid A deposition without any detection of cutaneous IgGl as the myeloma-derived paraprotein assumed to be causative for the development of systemic AA amyloidosis.
在一名患有IgG λ亚型多发性骨髓瘤的86岁女性中，同时出现了系统性淀粉样变性，表现为巨舌、弥漫性脱发和全身性皮肤受累。皮肤出现粟丘疹样丘疹、瘀点出血，组织脆性增加，随后形成水疱。典型的组织学和免疫组织学模式显示真皮内有大量淀粉样物质团块，与抗淀粉样蛋白A抗体呈阳性反应，其周围环绕着袖口样扩张的毛细血管。这些淀粉样沉积物的大量存在导致毛细血管和真皮基质明显失去弹性和脆性，最终导致出血性大疱性皮疹。本病例的独特之处在于，由于淀粉样蛋白A沉积导致的出血性大疱性皮肤损伤的强度，而未检测到皮肤IgG1，而骨髓瘤衍生的副蛋白被认为是系统性AA淀粉样变性发展的病因。
