Cutaneous porphyrias

Porphyria (refers to a group of disorders with high level of porphyrin in your body. Porphyrins are essential for the function of hemoglobin of red blood cells. Porphyria can affect the nervous sytem or the skin (cutaneous porphyrias). If you have cutaneous porphyrias, you are sensitive to sunlight. If you are exposed to sunlight, you will have sunburn, skin redness, skin pain, blisters, itchness, excessive hair growth in affected areas, and red or brown urine. Treatment includes reducing sunlight exposure, periodically drawing blood to reduce iron in your body in order to reduce porphyrins. Drugs used to treat malaria, hydroxychloroquine (Plaquenil) or chloroquine (Aralen), can absorb excess porphyrins. Since you avoid sunlight, vitamin D dietary supplement is needed.


How is Cutaneous porphyrias treated in practice?