Paller A S, Fine J D, Kaplan S, Pearson R W
Arch Dermatol. 1986 Jun;122(6):704-10.
We encountered four patients in the United States with the generalized atrophic benign form of junctional epidermolysis bullosa (epidermolysis bullosa atrophicans generalisata mitis, nonlethal junctional epidermolysis bullosa). Prior to the performance of definitive diagnostic studies, each patient had been thought for at least a decade to have either a dystrophic or simplex form of epidermolysis bullosa. Each patient had generalized blisters since birth that healed with atrophy and mild scarring but without milia or contractures. Two of the four patients had experienced laryngeal involvement during childhood. In each patient, correct diagnosis was finally established by either electron microscopic examination or immunofluorescence mapping of skin sections from induced blisters.
我们在美国遇到了4例泛发性萎缩性良性交界性大疱性表皮松解症（泛发性轻型萎缩性大疱性表皮松解症，非致死性交界性大疱性表皮松解症）患者。在进行确定性诊断研究之前，每位患者至少有十年时间被认为患有营养不良型或单纯型大疱性表皮松解症。每位患者自出生起就有全身性水疱，水疱愈合后出现萎缩和轻度瘢痕形成，但无粟丘疹或挛缩。4例患者中有2例在儿童期出现喉部受累。通过对诱导水疱的皮肤切片进行电子显微镜检查或免疫荧光定位，最终确诊了每位患者的病情。
