Yi S, Naito M, Takahashi K, Nogami R, Maekawa Y, Arao T
Second Department of Pathology, Kumamoto University Medical School, Japan.
Pathology. 1988 Apr;20(2):184-7. doi: 10.3109/00313028809066631.
An autopsy case of dystrophic epidermolysis bullosa, recessive type, complicated by systemic secondary amyloidosis is described. The patient had developed multiple bullous lesions and erosions from birth, followed by repeated infection. At autopsy, chronic persistent inflammation was observed in the skin and in various visceral organs, accompanied by systemic amyloidosis. By the peroxidase-antiperoxidase (PAP) method, amyloid deposits stained positively for anti-AA-protein antiserum. In the present case, we concluded that the systemic amyloidosis was of the AA type, and developed secondarily to the chronic persistent inflammation in the prolonged course of dystrophic epidermolysis bullosa, recessive type.
本文描述了1例隐性遗传性营养不良型大疱性表皮松解症并发系统性继发性淀粉样变的尸检病例。患者自出生起即出现多发性水疱性皮损及糜烂，随后反复感染。尸检发现皮肤及多个内脏器官存在慢性持续性炎症，并伴有系统性淀粉样变。通过过氧化物酶-抗过氧化物酶（PAP）法检测，淀粉样沉积物对抗AA蛋白抗血清呈阳性染色。在本病例中，我们得出结论，系统性淀粉样变为AA型，是在隐性遗传性营养不良型大疱性表皮松解症的长期病程中，继发于慢性持续性炎症而形成的。
