Porphyria turcica. Twenty years after hexachlorobenzene intoxication

Abstract

Porphyria involving more than 4000 persons occurred in Eastern Turkey from 1956-1961 due to ingestion of hexachlorobenzene (HCB), a fungicide added to wheat seedlings. During a site visit in 1977, 32 porphyric Turks (mean age, 29 yr) were examined. Porphyrin concentrations were determined with samples of urine and stool from 29 porphyric and 20 control Turks and compared with results from 40 subjects in the USA. Distinctive clinical features were found in porphyric subjects, including hyperpigmentation, hirsutism, severe scarring, short stature, pinched facies, small hands, painless arthritis, weakness and enlarged thryoids. Porphyrin levels were still substantially elevated in 5 subjects. Hexachlorobenzene,which is fat soluble, was detected in maternal milk of 1 porphyric patient and in 1 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 of pembe yara (pink sore), in which many children who were breast-fed by affected mothers died with associated weakness, convulsions and annular erythema.