Tsukazaki N, Okada S, Yoshida H
Department of Dermatology, Nagasaki University School of Medicine.
Nihon Rinsho. 1995 Jun;53(6):1463-7.
Examination was made of sclerodermoid changes in porphyria cutanea tarda (PCT) and PCT in association with true scleroderma. Although sclerodermoid change in PCT is rare in Japan, it is important in the diagnosis of PCT when there are few other clinical signs characteristic of PCT. In the present case, slight improvement in cutaneous sclerosis was noted following treatment for PCT, but pulmonary changes somewhat intensified. The reason for the association of PCT with progressive systemic sclerosis (PSS) remains obscure. Elevated uroporphyrin due to PCT may possibly increase cutaneous sclerosis in PSS. A diagnosis of PCT should be considered for patients presenting scleroderma, since it is difficult to distinguish sclerodermoid changes from true scleroderma.
对迟发性皮肤卟啉病（PCT）及合并真性硬皮病的PCT中的硬皮病样改变进行了检查。尽管PCT中的硬皮病样改变在日本很少见，但当PCT的其他临床特征性体征较少时，其对PCT的诊断很重要。在本病例中，PCT治疗后皮肤硬化有轻微改善，但肺部改变有所加重。PCT与进行性系统性硬化症（PSS）相关的原因仍不清楚。PCT导致的尿卟啉升高可能会增加PSS中的皮肤硬化。对于出现硬皮病的患者应考虑PCT的诊断，因为很难将硬皮病样改变与真性硬皮病区分开来。
