caa a22 4500 465774008 CHVBK 20180323111936.0 cr unu---uuuuu 170327e19900101xx s 000 0 eng 10.1007/BF00493468 doi (NATIONALLICENCE)springer-10.1007/BF00493468 Skin changes in variegate porphyria [Elektronische Daten] Clinical, histopathological, and ultrastructural study [K. Timonen, K. Niemi, P. Mustajoki, R. Tenhunen] Summary: The skin of 20 patients with variegate porphyria (VP) was studied using light, fluorescent, and electron microscopy. Twelve patients had skin symptoms and markedly increased fecal protoporphyrin excretion. Their sun-exposed skin was characterized by homogeneous PAS-positive thickening and IgG deposition in the vessel walls. The basic ultrastructural change was thickening of the vascular walls caused by reduplication of the basal lamina and perivascular deposition of amorphous material. Qualitatively similar but less prominent histopathological changes occurred in sun-protected skin in some of the patients. Six patients had no skin symptoms but an increased porphyrin excretion. The light microscopical changes were comparable to those in the patients with skin symptoms, but the ultrastructural changes were less severe. No abnormal histopathological changes occurred in two symptomless patients who had low lymphocyte protoporphyrinogen oxidase activity but normal fecal porphyrin excretion. These results show that the primary site of skin damage in VP is the vessel wall, and that histopathological changes of the skin also occur in porphyric patients who have never had skin symptoms. Factors determining the occurrence of skin symptoms in VP are discussed. Springer-Verlag, 1990 Porphyria nationallicence Dermal vessels nationallicence Ultrastructure nationallicence Timonen K. Department of Dermatology, University Central Hospital, University of Helsinki, Snellmaninkatu 14, SF-00170, Helsinki, Finland aut Niemi K. Department of Dermatology, University Central Hospital, University of Helsinki, Snellmaninkatu 14, SF-00170, Helsinki, Finland aut Mustajoki P. Third Department of Medicine, University of Helsinki, Helsinki, Finland aut Tenhunen R. Department of Clinical Chemistry, University of Helsinki, Helsinki, Finland aut Archives of Dermatological Research Springer-Verlag 282/2(1990-01-01), 108-114 0340-3696 282:2<108 1990 282 403 https://doi.org/10.1007/BF00493468 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF00493468 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Timonen K. Department of Dermatology, University Central Hospital, University of Helsinki, Snellmaninkatu 14, SF-00170, Helsinki, Finland aut NATIONALLICENCE 700 1- Niemi K. Department of Dermatology, University Central Hospital, University of Helsinki, Snellmaninkatu 14, SF-00170, Helsinki, Finland aut NATIONALLICENCE 700 1- Mustajoki P. Third Department of Medicine, University of Helsinki, Helsinki, Finland aut NATIONALLICENCE 700 1- Tenhunen R. Department of Clinical Chemistry, University of Helsinki, Helsinki, Finland aut NATIONALLICENCE 773 0- Archives of Dermatological Research Springer-Verlag 282/2(1990-01-01), 108-114 0340-3696 282:2<108 1990 282 403 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer