Brajac Ines, Kastelan Marija, Gruber Franjo, Peris Zdravko
Department of Dermatology, University Hospital Centre Rijeka, Kresimirova 42, 51000 Rijeka, Croatia.
Eur J Dermatol. 2004 Sep-Oct;14(5):310-3.
Hyalinosis cutis et mucosae is an inherited disease, characterized by deposition of a hyaline-like substance in the dermis and internal organs. In the present study, we report a 30 year follow up in a female patient with hyalinosis cutis et mucosae treated using dermabrasion and oral retinoids. In our opinion, the lesion on uncovered body areas should be corrected as soon as possible to enable a normal psychophysical development. The lesions on skin and oral mucosa may be successfully removed without remission with dermabrasion in early childhood. In addition, oral retinoids in adulthood may produce some improvement.
皮肤黏膜透明变性是一种遗传性疾病,其特征为在真皮和内脏器官中沉积一种透明样物质。在本研究中,我们报告了一名接受磨皮术和口服维甲酸治疗的皮肤黏膜透明变性女性患者的30年随访情况。我们认为,应尽快矫正暴露身体部位的病变,以实现正常的心理生理发育。在儿童早期,通过磨皮术可成功去除皮肤和口腔黏膜上的病变且无复发。此外,成年后口服维甲酸可能会有一定改善。