Dermatology pp 899-912 | Cite as

Porphyrias

  • Otto Braun-Falco
  • Gerd Plewig
  • Helmut H. Wolff
  • Richard K. Winkelmann

Abstract

Porphyrias are rare inborn or acquired diseases related to disorders in heme biosynthesis. This type of defect takes various clinical forms characterized by elevated contents of certain porphyrins or their precursors in the tissues, and urine, or stool. The porphyrins are light-sensitizing (phototoxic) substances, and disorders related to them result in phototoxic symptoms in skin areas exposed to light. Only acute intermittent porphyria is an exception. The genetically conditioned biochemical anomaly is not obvious in some patients, one therefore speaks of latent porphyria. In such cases the disease is sometimes made manifest by provocation, e.g., by drugs such as chloroquine, griseofulvin, or oral contraceptives, as well as by chemicals, environmental pollutants, or heavy metals.

Synonyms

Porphyria erythropoietica congenita Günther’s disease congenital porphyria. Protoporphyria erythropoietica etythrohepatic protoporphyria protoporphyrinemic light dermatosis. Porphyria acuta intermittens porphyria hepatica acuta. Porphyria vaiegata hereditary pootoco-proproporphyria mixed hepatic porphyria South African genetic porphyria. Porphyria hepatica chronica chronic porphyria syndrome actinic-traumatic bullous porphyrin dermatosis(Tappeiner 1953) porphyria bullosa congenita tarda. Porphyria hepatoerythrocytaria erythropoietic porphyrin-hepatitis. 

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Copyright information

© Springer-Verlag Berlin Heidelberg 1991

Authors and Affiliations

  • Otto Braun-Falco
    • 1
  • Gerd Plewig
    • 2
  • Helmut H. Wolff
    • 3
  • Richard K. Winkelmann
    • 4
  1. 1.Dermatologische Klinik und PoliklinikLudwig-Maximilians-Universität MünchenMünchen 2Federal Republic of Germany
  2. 2.Hautklinik der Heinrich-Heine-UniversitätDüsseldorfFederal Republic of Germany
  3. 3.Klinik für Dermatologie und VenerologieMedizinischen Universität zu LübeckLübeck 1Federal Republic of Germany
  4. 4.Dermatology — Dermatopathology, Mayo ClinicMayo Medical SchoolRochesterUSA

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