Murphy C M, Grau-Massanés M, Sánchez R L
University of Texas Medical Branch, Galveston 77555-0783, USA.
J Cutan Pathol. 1995 Dec;22(6):556-62. doi: 10.1111/j.1600-0560.1995.tb01151.x.
An 11-year-old hispanic female was referred for evaluation of multiple asymptomatic papules which had slowly developed since age two. The patient had no significant medical history and no family members, including an older sibling, were similarly affected. Physical examination revealed multiple 2-6 mm flesh-colored to erythematous papules over the posterior scalp, trunk, and extremities, predominantly in a midline distribution. No lentigines, blue nevi, or evidence of endocrine ab-normalities were noted. Echocardiogram was normal with no evidence of myxoma. Six cutaneous biopsies showed myxomas, as evidenced by well-circumscribed dermal tumors composed of stellate, dendritic cells embedded in a mucinous stroma. Special stains and electron microscopy confirmed the H&E finding. Immunoperoxidase staining for Factor XIIIa was positive in the dendritic tumor cells, suggesting a relationship with dermal dendrocytes. This patient represents a case of multiple cutaneous myxomas without other components of Carney's complex of myxomas, spotty pigmentation, endocrine overactivity, and a variety of uncommon tumors. A long-term follow-up is indicated since some of the above cited elements of the syndrome could appear over time.
一名11岁的西班牙裔女性因自两岁起逐渐出现的多个无症状丘疹前来接受评估。该患者无重大病史,包括年长的兄弟姐妹在内的家庭成员也未出现类似症状。体格检查发现后头皮、躯干和四肢有多个2 - 6毫米大小、肤色至红斑样的丘疹,主要呈中线分布。未发现雀斑、蓝痣或内分泌异常迹象。超声心动图正常,未发现黏液瘤迹象。六次皮肤活检显示为黏液瘤,表现为边界清晰的真皮肿瘤,由嵌入黏液基质中的星状、树枝状细胞组成。特殊染色和电子显微镜检查证实了苏木精 - 伊红染色的结果。免疫过氧化物酶染色显示树枝状肿瘤细胞中因子XIIIa呈阳性,提示与真皮树突状细胞有关。该患者代表了一例多发性皮肤黏液瘤病例,不伴有卡尼综合征的其他成分,如黏液瘤、斑点状色素沉着、内分泌功能亢进以及多种罕见肿瘤。由于该综合征的上述一些症状可能会随时间出现,因此需要进行长期随访。