Porphyria turcica due to hexachlorobenzene: a 20 to 30 year follow-up study on 204 patients

Abstract

During 1955-1961 in south-east Turkey, over 3000 patients developed porphyria due to ingestion of hexachlorobenzene, a fungicide added to wheat seedlings. Subsequently they developed pigmentation, hirsutism, weakness, porphyrinuria and bullae. The condition was called kara yara or 'black sore'. Many of the breast-fed children under the age of 1 year died from a disease known as pembe yara or 'pink sore'. In this follow-up study of 204 patients, 20-30 years later, there were 132 males and 72 females, average age 32.1 years (at time of examination), with an average age of onset of 10.2 years, and duration of 2.4 years. Neurological, dermatological, and orthopaedic abnormalities still persisted. Neurological symptoms included weakness (66%), paraesthesiae (54%), neuritis (63%), myotonia (49%), and occasional 'cogwheeling' (29%). Some individuals affected before puberty had small stature (44%), small hands (64%) and painless arthritis (67%). Severe residual scarring was common (85%), and pinched facies (42%), hirsutism (47%) and hyperpigmentation (71%) were also observed. Enlarged thyroids were observed in 37% (59% in females), which was above average of 5% in this area of Turkey. Porphyrin excretion, particularly uroporphyrin in the urine and stools, was still significantly increased in seventeen patients. Hexachlorobenzene levels as high as 2.8 p.p.m. were detected in human milk, and the average level (0.29 p.p.m. +/- s.d. 0.06) was 140 times the level allowed in cow's milk.