Yeo Y W, Oon H H, Lee J S, Pan J Y, Mok Y J, Ng S K
Singapore General Hospital.
Dermatol Online J. 2016 Apr 18;22(4):13030/qt9qq3d88q.
Acral pseudolymphomatous angiokeratoma of children (APACHE) is a rare form of cutaneous pseudolymphoma characterized byangiomatous papules with a predilection for the acral regions of children. Classically, a dense dermal lymphocytic infiltrate composed of both T and B cells is seen in histological specimens, together with prominent vessels lined by plump endothelial cells. Increasing evidence suggests that this condition is neither necessarily acral, pseudolymphomatous, nor angiokeratomatous. It may not always be a pediatric disease. Therefore, the correctness of its nomenclature has been questioned. Herein, we report threecases whose clinical and histological features were consistent with the diagnosis of APACHE. To our knowledge, this is the first report of APACHE from Southeast Asia. We also discuss why we believe "APACHE" to be a misnomer and support "papular angiolymphoid hyperplasia" as a more accurate and encompassing term. In addition, we illustrate a case with significant overlapping features with lymphoplasmacytic plaque in children, suggesting that both entities may exist on a clinical andhistological spectrum.
儿童肢端假性淋巴瘤性血管角皮瘤(APACHE)是一种罕见的皮肤假性淋巴瘤,其特征为血管性丘疹,好发于儿童的肢端部位。典型情况下,组织学标本可见由T细胞和B细胞组成的致密真皮淋巴细胞浸润,以及由丰满内皮细胞衬里的明显血管。越来越多的证据表明,这种疾病不一定局限于肢端,也不一定是假性淋巴瘤性或血管角皮瘤性的。它可能并不总是一种儿科疾病。因此,其命名的正确性受到了质疑。在此,我们报告三例临床和组织学特征与APACHE诊断相符的病例。据我们所知,这是东南亚地区关于APACHE的首例报告。我们还讨论了为什么我们认为“APACHE”是一个不恰当的名称,并支持“丘疹性血管淋巴样增生”作为一个更准确、涵盖性更强的术语。此外,我们展示了一例与儿童淋巴浆细胞性斑块有显著重叠特征的病例,提示这两种疾病在临床和组织学上可能存在谱系关系。