Marrero Alemán Gabriel, El Habr Constantin, Islas Norris Diana, Montenegro Dámaso Társila, Borrego Leopoldo, Sangueza Omar P
*Department of Dermatology, Complejo Hospitalario Universitario Insular Materno-Infantil, Universidad de Las Palmas de Gran Canaria, Las Palmas de Gran Canaria, Spain;†Department of Pathology and Dermatology, Wake Forest Baptist Health, Winston-Salem, NC; and‡Department of Pathology, Complejo Hospitalario Universitario Insular Materno-Infantil, Las Palmas de Gran Canaria, Spain.
Am J Dermatopathol. 2017 Apr;39(4):310-312. doi: 10.1097/DAD.0000000000000768.
Cutaneous mastocytosis is defined by the presence of mast cells within the skin in the absence of other criteria for the diagnosis of systemic mastocytosis. Mast cells are characterized by an abundant granular cytoplasm and a round to oval or spindle-shaped nuclei. The presence of mast cells with bilobed and multilobed nuclei in cutaneous mastocytosis is a rare phenomenon and has been rarely reported in the literature. To our knowledge, there are only 4 reported cases of cutaneous mastocytosis with atypical mast cells. We hereby report a case of urticaria pigmentosa in a 7-year-old female patient. The patient presented with asymptomatic skin lesions of several years duration over the neck and left scapular area. Histopathological examination revealed the presence of middermal perivascular infiltrates mainly composed of mast cells, few lymphocytes, and eosinophils. Most mast cells showed pleomorphic nuclei with bilobed and multilobed morphology that revealed a positive expression for CD117, tryptase, CD68, and Giemsa stains. Based on these findings the diagnosis of urticaria pigmentosa with atypical mast cells was made. Additional tests to rule out systemic involvement were performed. All values, including a tryptase level, were within normal limits. No changes were noted after 1-year follow-up.
皮肤肥大细胞增多症的定义是皮肤内存在肥大细胞，且不存在系统性肥大细胞增多症的其他诊断标准。肥大细胞的特征是细胞质富含颗粒，细胞核呈圆形至椭圆形或梭形。在皮肤肥大细胞增多症中出现具有双叶和多叶核的肥大细胞是一种罕见现象，文献中鲜有报道。据我们所知，仅有4例关于具有非典型肥大细胞的皮肤肥大细胞增多症的报道。我们在此报告一例7岁女性患者的色素性荨麻疹。该患者颈部和左肩胛区出现持续数年的无症状皮肤病变。组织病理学检查显示真皮中层血管周围浸润，主要由肥大细胞、少量淋巴细胞和嗜酸性粒细胞组成。大多数肥大细胞显示出具有双叶和多叶形态的多形核，对CD117、类胰蛋白酶、CD68和吉姆萨染色呈阳性表达。基于这些发现，做出了伴有非典型肥大细胞的色素性荨麻疹的诊断。进行了排除系统性受累的其他检查。所有指标，包括类胰蛋白酶水平，均在正常范围内。1年随访后未见变化。
