Tukenmez Demirci Gulsen, Altunay Ilknur Kıvanc, Mertoglu Eda, Kucukunal Aslı, Sakız Damlanur
Sisli Etfal Education and Training Hospital, Dermatology Department, Sisli/Istanbul, Turkey.
J Dermatol Case Rep. 2011 Sep 21;5(3):47-9. doi: 10.3315/jdcr.2011.1074.
Linear atrophoderma is a rare disease, first described by Moulin in 1992 in 5 patients. It is an acquired unilateral hyperpigmented, depressed band-like areas following the lines of Blaschko. It affects children or adolescents of both genders involving the trunk or the limbs. It is considered to be a rare cutaneous form of mosaicism.
A 39-year-old woman with a 22 years history of unilateral slightly depressed hyperpigmented lesion on her neck was admitted to us. The skin texture was normal and there were no signs of induration or sclerosis. The histopathological examination revealed a normal epidermis outlined by a hyperpigmented basal layer. In the papillary dermis proliferation of superficial vessels with mild lymphocytic infiltrate and melanin-laden macrophages were present. The collagen fibres and elastic fibres were normal. The clinical and histopathological features confirmed the diagnosis of linear atrophoderma of moulin. We discussed the case according to the other cases reported in the literature.
Approximately 28 cases of linear atrophoderma have been reported in literature. The present case has the charecteristic clinical and histopathological features of linear atrophoderma as defined by Moulin, but the localization of the lesion is unusual.
线状皮肤萎缩症是一种罕见疾病，1992年由穆兰首次描述，当时有5例患者。它是一种后天性的单侧色素沉着、沿布拉斯科线分布的凹陷带状区域。它影响男女儿童或青少年，累及躯干或四肢。它被认为是一种罕见的皮肤嵌合形式。
一名39岁女性因颈部有22年单侧轻度凹陷性色素沉着病变病史入院。皮肤质地正常，无硬结或硬化迹象。组织病理学检查显示表皮正常，基底层色素沉着。在乳头真皮层，有浅表血管增生，伴有轻度淋巴细胞浸润和含黑色素巨噬细胞。胶原纤维和弹性纤维正常。临床和组织病理学特征确诊为穆兰线状皮肤萎缩症。我们根据文献报道的其他病例讨论了该病例。
文献中报道了约28例线状皮肤萎缩症病例。本病例具有穆兰所定义的线状皮肤萎缩症的特征性临床和组织病理学特征，但病变部位不寻常。
