Mitre Victoria, Applebaum Danielle S, Albahrani Yasser, Hsu Sylvia
Baylor College of Medicine, Department of Dermatology, Houston, Texas.
Dermatol Online J. 2017 Jul 15;23(7):13030/qt25v009gs.
Fixed drug eruption (FDE) is defined as sharply demarcated erythematous patches or plaques that occur secondary to systemic exposure to a causative medication. Eruptions are deemed "fixed" because upon repeated exposure they recur at previously affected sites. Generalized bullous fixed drug eruption (GBFDE) is a rare FDE variant occurring in patients with a previous history of FDE. Given the extensive cutaneous involvement and the frequent mucosal ulcerations associated with GBFDE, it is challenging to discern these lesions from Steven-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). The presence of significantly elevated lesional and serum granulysin in SJS/TEN is an important discriminating factor because granulysin levels remain significantly lower in GBFDE. The implementation of an immunochromatographic test for rapid detection of elevated granulysin levels could therefore facilitate the early diagnosis of SJS/TEN. We report a case of GBFDE to elucidate the characteristic differences in clinical presentation, histopathology, and immunohistochemistry that can facilitate diagnosis.
固定性药疹（FDE）被定义为因全身接触致病药物而继发出现的边界清晰的红斑性斑片或斑块。皮疹被认为是“固定的”，因为在再次接触时，它们会在先前受累部位复发。泛发性大疱性固定性药疹（GBFDE）是一种罕见的FDE变异型，发生于有FDE病史的患者。鉴于GBFDE常伴有广泛的皮肤受累和频繁的黏膜溃疡，将这些皮损与史蒂文斯-约翰逊综合征（SJS）和中毒性表皮坏死松解症（TEN）相鉴别具有挑战性。SJS/TEN中皮损和血清颗粒溶素水平显著升高是一个重要的鉴别因素，因为GBFDE中的颗粒溶素水平仍显著较低。因此，开展免疫层析试验以快速检测升高的颗粒溶素水平有助于SJS/TEN的早期诊断。我们报告1例GBFDE病例，以阐明有助于诊断的临床表现、组织病理学和免疫组化方面的特征性差异。
