Krajnc I, Vizjak A, Hvala A, Jurcić V, Rozman B
Medical Department-Rheumatology, Maribor Teaching Hospital, Maribor.
Wien Klin Wochenschr. 1998 Oct 2;110(18):651-4.
Porphyria cutanea tarda (PCT) is one of several entities in the differential diagnosis of scleroderma. We report a 62-year-old man with PCT diagnosed since two decades. Clinical data and the results of light microscopy, immunohistochemistry, immunofluorescence and electron microscopy of skin biopsies are presented. The biopsy revealed sclerosis of the dermis mainly due to increased collagens I and III, and accumulation of collagen IV, which had caused the vessel wall to thicken. Immunofluorescence for detection of immune reactants was negative. It was concluded that the histomorphology of PCT of long duration may be similar to that of scleroderma. Nevertheless, in addition to clinical findings, detailed skin biopsy studies including contemporary techniques can contribute to the differentiation of these diseases.
迟发性皮肤卟啉病（PCT）是硬皮病鉴别诊断中的几种病症之一。我们报告一名62岁男性，二十年前被诊断为PCT。本文呈现了临床数据以及皮肤活检的光镜、免疫组化、免疫荧光和电镜检查结果。活检显示真皮硬化，主要原因是I型和III型胶原蛋白增加以及IV型胶原蛋白积聚，这导致血管壁增厚。检测免疫反应物的免疫荧光为阴性。结论是，长期PCT的组织形态学可能与硬皮病相似。然而，除了临床发现外，包括现代技术在内的详细皮肤活检研究有助于这些疾病的鉴别诊断。
