Wednesday, November 22, 2017

Red-producing protoporphyria.. The appearance of urinary lesions and even vesicles or bubbles on areas exposed to the sun

Red-producing protoporphyria:
This form appears early in childhood from 2 to 5 years and is inherited as a compelling feature. Photodynamic susceptibility is a distinctive protoformuria-generated appearance of red.
In this form, it is thought to be caused by longer wavelengths of UVL, which range from 320-450 nm. It should be noted that even glass window does not give protection and protection from the impact of the sun on these patients.
Clinical manifestations:
- Skin lesions are an edema of the aortic shape of the erythema.
- The appearance of pests and even villains or bubbles on areas exposed to the sun.
- Skin lesions may be cured with scarring and with waxy thymic in the nose and cheeks, and above the proximal joints.
- Atrophy and scarring around the mouth.
Diagnosis methods:
- Form and distribution of skin lesions.
- Hypersensitivity.
- increase porphyrin proto porphyrin and copropor furine in the stool.
- Blood plasma microscopy: A few drops of blood are resolved by 1: 5 of the physiological serum and placed on the microscope slide and examined with the submersible lens in the microscope.
- Red erythrocyte tend to have a distinctive sensation.
- It should be noted that in this form of urveria, urine is not usually given as a fluorescence under the rays of wood.