Friday, Jun. 21, 1963
The Night People
While nearly everyone else strips down to tan, Mrs. Norton J. Carlson, 59, of Grand Junction, Colo., covers up for safety. For her, no suntan lotion the chemists can devise is ever likely to be good enough. When Mrs. Carlson set out on a 342-mile auto trip to visit her sister a few weeks ago, it was like minor royalty fleeing restless natives. She waited for nightfall in the shadows of her parlor. Then she put on a dress with extra-long skirt and sleeves, pulled up her gloves, wrapped a kerchief about her face, and stepped nervously into a waiting car with tinted windows. All such precautions, Mrs. Carlson has learned from agonizing experience, are absolutely essential. She suffers from a form of the rare disease porphyria, and to venture into the daylight unprotected for so much as a few seconds causes painful skin eruptions.
The strange sickness is incurable and not fully understood. Through an inborn metabolic quirk, the body produces an excess of porphyrins, chemicals that are usually produced only in tiny amounts and seem somehow to be involved with the body's sensitivity to the sun. In some forms of porphyria, skin sensitivity is slight, but the victim suffers severe abdominal pains, bizarre mental disturbances, and sometimes respiratory paralysis. Mrs. Carlson suffers from a form called porphyria cutaneatarda, in which the porphyrin overproduction can be traced to an inherited liver malfunction. Doctors have studied such cases for years, but have only recently found a strong clue to what actually happens in the body.
The new knowledge is interesting; but to Mrs. Carlson, it means little that two Harvard Medical School researchers studied porphyria patients to find out whether a major change in the porphyrin content of their skin takes place before or after exposure to sunlight. Dr. J. W. Burnett and Dr. M. A. Pathak examined two victims and three healthy subjects, both after long confinement indoors and after exposure to the sun. In the people with porphyria, output of porphyrin compounds rose sharply after exposure to the light; the others showed no change. Sunlight, the doctors concluded, increases the concentration of porphyrin in the skin and red blood cells. But how the excess porphyrin does its damage, they still cannot say.
Ultraviolet light, the Harvard researchers report in the New England Journal of Medicine, produces photo-sensitization by activating the porphyrin already present in the blood and skin. They suspect that it also increases the production of porphyrin. This is more than medicine has known before about the elusive disease, but it is still far from suggesting a cure. For Mrs. Carlson and her fellow sufferers, the only prescription remains to avoid sunlight like the plague.
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