Late skin porphyria is the most common type of porphyria that causes small lesions to appear on skin exposed to the sun, such as the back of the hand, face or scalp, due to the lack of an enzyme produced by the liver that leads to the accumulation of iron in the skin. blood and skin. Cutaneous porphyria has no cure, but it can be controlled with the use of medicines prescribed by the dermatologist.
Generally, late skin porphyria appears during adulthood, especially in patients who frequently drink alcohol or who have liver problems, such as hepatitis C, for example.
Late skin porphyria is usually not genetic, however, in some cases it can pass from parents to children, and genetic counseling is recommended before becoming pregnant, if there are several cases in the family.
Symptoms of cutaneous porphyria
The first symptom of cutaneous porphyria is the appearance of small blisters on the skin exposed to the sun, which take time to heal, however, other symptoms include:
- Exaggerated growth of hair on the face; Hardened skin in some places, such as arms or face; Darkened urine.
After the blisters disappear, scars or light spots may appear that take a long time to heal.
The diagnosis of cutaneous porphyria must be made by a dermatologist through blood, urine and feces tests to confirm the presence of porphyrin in the cells, as it is a substance produced by the liver during the disease.
Treatment for cutaneous porphyria
Treatment for cutaneous porphyria must be guided by a dermatologist in collaboration with a hepatologist, since it is necessary to control the levels of porphyrin produced by the liver. Thus, depending on the patient's symptoms, treatment can be done with remedies for cutaneous porphyria, such as chloroquine or hydroxychloroquine, regular blood withdrawal to decrease iron levels in cells or a combination of both.
In addition, during treatment it is recommended that the patient avoid alcohol consumption and sun exposure, even with sunscreen, and the best way to protect the skin from the sun is to wear pants, long-sleeved shirts, hat and gloves, for example.
