Department of Dermatology, University Complex Hospital of Vigo, Spain.
Indian J Dermatol Venereol Leprol. 2011 Mar-Apr;77(2):184-7. doi: 10.4103/0378-6323.77460.
The vast majority of mastocytosis appear in childhood, urticaria pigmentosa (UP) and mastocytomas being the most common types. Terms such as "xanthelasmoid mastocytosis", "pseudoxanthomatous mastocytosis" or "nodular mastocytosis" have been introduced in the literature to describe the presence of yellowish papular or nodular lesions. We describe two children with cutaneous mastocytosis showing yellowish lesions in combination with other skin lesions. A 10-year-old girl presented with asymptomatic lesions in her vulva at birth, and developed brownish macules on her trunk years after. An eight-year-old boy presented with multiple yellowish papular lesions on his trunk, neck and limbs coexisting with a few clinically anetodermic lesions. No systemic involvement was found and the skin biopsy confirmed a cutaneous mastocytosis in both cases. The two patients are currently asymptomatic and are being periodically followed up. Mastocytoses may show a variety of clinical lesions, sometimes leading to misdiagnosis. Although there are previous reports, involvement of the mucosae and secondary anetoderma are not common findings in cutaneous mastocytoses. We consider that cutaneous manifestations of mastocytoses compose a clinical spectrum, thus explaining the coexistence of different clinical lesions and the development of uncommon presentations.
绝大多数肥大细胞增多症出现在儿童期，其中蕈样肉芽肿（UP）和肥大细胞瘤是最常见的类型。文献中引入了“黄瘤样肥大细胞增多症”、“假性黄瘤样肥大细胞增多症”或“结节性肥大细胞增多症”等术语来描述黄色丘疹或结节性病变的存在。我们描述了两名患有皮肤肥大细胞增多症的儿童，他们的皮肤出现黄色病变，并伴有其他皮肤病变。一名 10 岁女孩出生时外阴部出现无症状病变，数年后躯干出现褐色斑疹。一名 8 岁男孩躯干、颈部和四肢出现多个黄色丘疹性病变，同时伴有少数临床无皮病病变。未发现全身受累，皮肤活检均证实为皮肤肥大细胞增多症。这两名患者目前无症状，正在定期随访。肥大细胞增多症可能表现出多种临床病变，有时会导致误诊。尽管有先前的报告，但黏膜受累和继发性无皮病并不常见于皮肤肥大细胞增多症中。我们认为肥大细胞增多症的皮肤表现构成了一个临床谱，从而解释了不同临床病变的共存和罕见表现的发展。
