Abstract

Genodermatoses with a marked sun sensitivity are called photogenodermatoses. They are very rare diseases in which the photosensitivity is an early and constant symptom. The photosensitivity can be expressed as sun-induced eruptions of the exposed skin areas within hours and days, as light-induced rumor formation within years, or as both symptom complexes together. An enzymatic defect of known (xeroderma pigmentosum) or unknown character, impairing the defense against or the recovery from sun-induced skin damage, can be described as the result of a genetic defect (Table 1). In other examples (porphyrias), an inborn error of metabolism leads to accumulation of products that act as endogenous photosensitizers (Table 2). The main diseases following one or the other type of pathogenesis are presented as the main headings in this article.

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