Porphyria Turcica

Abstract

• Porphyria involving more than 4,000 persons occurred in Eastern Turkey from 1956 to 1961 due to ingestion of hexachlorobenzene (HCB), a fungicide added to wheat seedlings. During a site visit in 1977, 32 porphyric Turks (mean age, 29 years) were examined. Porphyrin concentrations were determined with samples of urine and stool from 29 porphyric and 10 control Turks and compared with results from 40 subjects in the United States. Distinctive clinical features were found in porphyric subjects, including hyperpigmentation, hirsutism, severe scarring, short stature, pinched facies, small hands, painless arthritis, weakness, and enlarged thyroids. Porphyrin levels were still substantially elevated in five subjects. Hexachlorobenzene, which is fat soluble, was detected in maternal milk of one porphyric patient and in one other patient's fat. The level of HCB was not considered sufficient to account for persistence of porphyria but may be a clue to the cause ofpembe yara(pink sore), in which many children who were breast-fed by affected mothers died with associated weakness, convulsions, and annular erythema. (Arch Dermatol116:46-50, 1980)