San Gallicano Dermatological Institute, IRCCS, Via Elio Chianesi 53, 00144 Rome, Italy.
Eur J Dermatol. 2009 Sep-Oct;19(5):469-73. doi: 10.1684/ejd.2009.0724. Epub 2009 Jun 5.
Two patients with a generalized, progressive dyschromatosis disorder are described and investigated as a model to study the role of fibroblast-derived mediators on skin pigmentation. The patients (father and daughter) had had a widespread hyperpigmentation since early life which then progressively worsened with the appearance of hyperpigmented macules, café-au-lait macules and freckles, also involving the lips, palms and soles, intermixed with small hypopigmented spots. These features resembled those of familial progressive hyperpigmentation (FPH). Histology revealed a normal epidermis with pronounced keratinocyte hyperpigmentation and the presence of dermal melanophages. Ultrastructural analysis showed basal and suprabasal keratinocytes enriched in melanosome complexes. Immunohistochemical staining displayed an increased expression of hepatocyte growth factor (HGF), stem cell factor (SCF) and keratinocyte growth factor (KGF) in fibroblast-like cells of the upper dermis in hyperpigmented lesions of both patients, compared to control healthy skin. Our data suggest that a persistent activation of fibroblasts abnormally stimulating melanocyte functions is involved in hyperpigmentation disorders.
描述并研究了两例患有广泛进行性色素失调症的患者，以此作为研究成纤维细胞衍生介质对皮肤色素沉着作用的模型。这两名患者（父女）从早期开始就出现了广泛的色素沉着过度，随后随着色素斑、咖啡牛奶斑和雀斑的出现而逐渐恶化，嘴唇、手掌和脚底也出现了色素沉着过度，同时伴有少量色素减退斑。这些特征类似于家族性进行性色素沉着过度（FPH）。组织学显示正常的表皮，伴有明显的角质形成细胞色素沉着，真皮中有黑素细胞。超微结构分析显示基底和超基底角质形成细胞富含黑色素体复合物。与正常健康皮肤相比，两名患者的色素沉着过度病变的真皮上层成纤维细胞样细胞中，肝细胞生长因子（HGF）、干细胞因子（SCF）和角质形成细胞生长因子（KGF）的表达增加。我们的数据表明，持续激活的成纤维细胞异常刺激黑素细胞功能参与了色素沉着过度障碍。
