Klinische Untersuchungen zur Augenbeteiligung bei Porphyria cutanea tarda

In 1980 we started a long-term study at our department examining 54 Patients with p.c.t. (42 male, 12 female). Out of these patients, 32 were re-examined (23 male, 9 female) in 1986. No ocular lesions were found that might be attributable to the underlying disease. Possible symptoms of p.c.t. are an increased filling of the conjunctival vessels, a decreased tear's formation, as well as brownish pigmentations in conjunctiva, in the lid margin. These changes occur in the anterior eye segment. At the fundus, a dry, in part side-different macular destruction and uncharacteristic peripheral retinal degeneration were found. The most prominent functional disorders are the acquired colour vision deficiency Verriest Type III and FSM Type I, respectively. It is based upon the small decrease of the visual acuity (relative central scotoma), the decline in immediate adaption and the macular destruction. Possible etiopathogenetical links between the underlying disease and the tapetoretinal degeneration are discussed. The morphological and functional eye lesions are limited and show hardly any progression.