Abstract

1. Numerous cases of porphyria of the "cutanea tarda" type were observed recently in the southeastern districts of Turkey. The toxic agents appear to have been fungicides, including two mercury compounds together with hexachlorobenzol which were added to seed wheat. The disease did not appear in peasants who did not consume this seed wheat. There were no indications of buckwheat toxicity; furthermore, this seed is not cultivated in Turkey.

Six of the affected patients were treated in our clinic.

2. Clinically the patients presented cachexia, bullae, hyperpigmentation and hypertrichosis, i.e., skin lesions of the cutanea tarda type of porphyria. There were no cases of splenomegaly, and no abdominal or neurologic signs.

3. The patients were found to have an organic and functional liver disease. No suprarenal cortical dysfunction was recorded. X-rays of the bones were normal. No signs of hemolysis were present. Bone marrow studies revealed slight normoblastic hyperplasia.

4. Urinary porphobilinogen was repeatedly found to be negative. Urinary and fecal uroporphyrin 1 and coproporphyin 111 were present in excess. The fecal excretion of coproporphyrin 111 was greater than that of uroporphyrin 1, whereas in the urine the relation was reversed. The bone marrow content of porphyrin was not increased. No fluorescence was recorded in the fresh unstained blood smears by fluorescence microscopy.

5. Our cases had the clinical and laboratory findings consistent with the cutanea tarda type of porphyria.

6. The advanced and severe alteration of our patients' porphyrin metabolism may not be reversible. Although the bullae disappeared during their hospital residence, the excessive porphyrin excretion was still present on their departure from the clinic.

7. For therapy, the patients were given a high caloric diet of protein rich food and treated with liver extracts, vitamin B1, B2, B12 and whole B-complex preparations. We can express no opinion as to the value of this treatment.

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