Cash Sarah H, Dever Tara T, Hyde Patrice, Lee Jason B
Department of Dermatology and Cutaneous Biology, Thomas Jefferson University Hospital, 833 Chestnut St, Ste 704, Philadelphia, PA 19107, USA.
Arch Dermatol. 2007 Sep;143(9):1164-7. doi: 10.1001/archderm.143.9.1164.
Large acquired melanocytic nevi that occur in patients with epidermolysis bullosa (EB), referred to as EB nevi, may pose a diagnostic challenge because of their clinical and dermoscopic resemblance to melanoma. These unconventional melanocytic nevi have been encountered in all categories of hereditary EB, most of them in childhood. Although some of the reported cases have an alarming clinical appearance that is indistinguishable from melanoma, long-term follow-up has confirmed the benign nature of these rarely encountered melanocytic lesions. The histopathologic patterns of these nevi range from a banal congenital pattern to the problematic persistent pseudomelanoma pattern.
We describe the clinical, dermoscopic, and histopathologic features of a large EB nevus in a toddler. Clinically, the lesion was markedly asymmetrical and irregularly pigmented with foci of stippled pigmentation and scarring, which easily fulfilled the ABCD criteria for melanoma. Accordingly, a false-positive score resulted when dermoscopy was performed. Histopathologically, a pattern of persistent melanocytic neoplasm was observed. In the following 18 months, dynamic changes of the lesion included near-complete disappearance of the pigment, which was replaced by scar, milia, and areas of healing ulcers.
Epidermolysis bullosa nevi are dynamic melanocytic lesions that may simulate melanoma.
大的获得性黑素细胞痣发生于大疱性表皮松解症(EB)患者,被称为EB痣,因其临床和皮肤镜表现与黑色素瘤相似,可能带来诊断挑战。这些非典型黑素细胞痣在所有遗传性EB类型中均有出现,多数在儿童期。尽管部分报道病例的临床表现令人担忧,与黑色素瘤难以区分,但长期随访证实这些罕见的黑素细胞病变为良性。这些痣的组织病理学模式从普通的先天性模式到有问题的持续性假黑色素瘤模式不等。
我们描述了一名幼儿身上一个大的EB痣的临床、皮肤镜和组织病理学特征。临床上,该病变明显不对称,色素沉着不规则,有散在的点状色素沉着和瘢痕,很容易符合黑色素瘤的ABCD标准。因此,进行皮肤镜检查时得出了假阳性结果。组织病理学上,观察到持续性黑素细胞肿瘤的模式。在接下来的18个月里,病变的动态变化包括色素几乎完全消失,被瘢痕、粟丘疹和愈合溃疡区域所取代。
大疱性表皮松解症痣是可能模拟黑色素瘤的动态黑素细胞病变。