Kolivras Athanassios, André Josette, Thompson Curtis, Sass Ursula, Fraitag Sylvie
Department of Dermatology and Dermatopathology, Saint-Pierre, Brugmann and HUDERF Hospitals, Université Libre de Bruxelles, Brussels, Belgium.
Department of Biomedical Engineering, Oregon Health Sciences University, Portland, OR, USA.
J Cutan Pathol. 2016 Apr;43(4):388-93. doi: 10.1111/cup.12651. Epub 2015 Dec 8.
Xanthelasmoid mastocytosis or xanthelasmoidea is a rare clinical variant of cutaneous mastocytosis characterized by a yellow hue of the clinical lesions, which are often misdiagnosed as juvenile xanthogranuloma. We present two pediatric cases of xanthelasmoid mastocytosis presenting as isolated mastocytomas, which are notable histopathologically for their hypervascularity. This pseudoangiomatous variant of cutaneous mastocytosis is important for pathologists to have knowledge of, so that a diagnosis of a vascular tumor is not rendered accidentally. The yellow hue has previously been explained by the usual deep and solid dermal mast cell infiltrate. In the two presented cases, however, the mast cell infiltrate was sparse, and the yellow color cannot be related to infiltrate density. We believe that the hypervascularity is at least one factor in the production of clinical xanthelasmoid appearance, and we propose the term 'pseudoangiomatous xanthelasmoid mastocytosis' to properly describe this rare variant of cutaneous mastocytosis.
睑黄瘤样肥大细胞增多症或睑黄瘤样病变是皮肤肥大细胞增多症的一种罕见临床变体，其临床病变具有黄色色调，常被误诊为幼年黄色肉芽肿。我们报告两例以孤立性肥大细胞瘤形式出现的睑黄瘤样肥大细胞增多症儿科病例，其组织病理学特征为血管丰富。皮肤肥大细胞增多症的这种假血管瘤样变体对于病理学家来说了解它很重要，这样就不会意外地诊断为血管肿瘤。黄色色调此前被解释为通常深部和实性的真皮肥大细胞浸润。然而，在这两个病例中，肥大细胞浸润稀疏，黄色与浸润密度无关。我们认为血管丰富至少是产生临床睑黄瘤样外观的一个因素，并且我们提出“假血管瘤样睑黄瘤样肥大细胞增多症”这一术语来恰当描述这种罕见的皮肤肥大细胞增多症变体。
