Hypersplenism with deficiency of platelets may occur. Fluorescence of teeth as well as of red blood cells and their precursors in the blood and bone marrow can be demonstrated by irradiation with the Soret band. As with other photosensitive porphyrias, window glass and conventional ultraviolet sunscreens are ineffective in blocking the offending wavelengths. Suppression of erythropoiesis and therefore heme synthesis can be achieved by transfusion and, when hemolysis is present, by splenectomy. Consequently, urinary excretion of uroporphyrin I and coproporphyrin I is reduced.
Treatment also includes avoidance of sunlight and topical care for skin lesions.
Oral sorbents such as cholestyramine and, more recently, charcoal have been advocated to decrease reabsorption of porphyrins from the intestine. Prompt and effective decreases of porphyrin concentrations in the urine, plasma, and skin and resolution of photosensitivity have been documented during therapy. Even with such treatment, however, progressive mutilation and premature death frequently occur.