Shah Kara N, Anderson Elizabeth, Junkins-Hopkins Jacqueline, James William D
Department of Dermatology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA.
Pediatr Dermatol. 2007 Nov-Dec;24(6):632-6. doi: 10.1111/j.1525-1470.2007.00552.x.
Medallion-like dermal dendrocyte hamartomas are rare congenital cutaneous lesions, with only three occurrences reported in the English language literature. They present at birth as asymptomatic circular, oval, or triangular well-circumscribed, atrophic patches. Typically, they have an erythematous or yellow-brown hue and a characteristic pliable, wrinkled surface; subtle telangiectases may also be appreciated. They may be misdiagnosed as atrophoderma, cutis aplasia, or anetoderma. All reported patients have been female. Characteristic histologic findings include epidermal atrophy and the presence of a CD34-positive spindle cell proliferation in the dermis. This spindle cell proliferation represents a population of dermal dendrocytes, which are bone marrow-derived cells that are believed to function as antigen-presenting cells that contribute to the function of the skin immune system. Little is known about the pathophysiology of medallion-like dermal dendrocyte hamartomas. We present a patient with this entity and review similar presentations reported in the literature.
奖章样真皮树突状细胞错构瘤是一种罕见的先天性皮肤病变，英文文献中仅报道过3例。它们在出生时表现为无症状的圆形、椭圆形或三角形边界清晰的萎缩性斑块。通常，它们有红斑或黄褐色色调，表面有特征性的柔韧、起皱；也可能可见细微的毛细血管扩张。它们可能被误诊为皮肤萎缩症、皮肤发育不全或皮肤松弛症。所有报道的患者均为女性。特征性组织学表现包括表皮萎缩以及真皮中存在CD34阳性梭形细胞增殖。这种梭形细胞增殖代表一群真皮树突状细胞，它们是骨髓来源的细胞，被认为作为抗原呈递细胞有助于皮肤免疫系统的功能。关于奖章样真皮树突状细胞错构瘤的病理生理学知之甚少。我们报告了1例患有该疾病的患者，并回顾了文献中报道的类似病例。
