EPP can also manifest between the ages of 3 and 6.Prolonged exposure to the sun can lead to edema of the hands, face, and feet, rarely with blistering and petechiae. Skin thickening can sometimes occur over time.People with EPP are also at increased risk to develop gallstones. One study has noted that EPP patients suffer from vitamin D deficiency. Liver failure
Protoporphyrin accumulates to toxic levels in the liver in 5–20% of EPP patients, leading to liver failure. The spectrum of hepatobiliary disease associated with EPP is wide. It includes cholelithiasis, mild parenchymal liver disease, progressive hepatocellular disease and end-stage liver disease.A lack of diagnostic markers for liver failure makes it difficult to predict which patients may experience liver failure, and the mechanism of liver failure is poorly understood. A retrospective European study identified 31 EPP patients receiving a liver transplant between 1983 and 2008, with phototoxic reactions in 25% of patients who were unprotected by surgical light filters. The same study noted a 69% recurrence of the disease in the grafted organ. Five UK liver transplants for EPP have been identified between 1987 and 2009. Frequent liver testing is recommended in EPP patients where no effective therapy has been identified to manage liver failure to date. Pregnancy
EPP photosensitivity symptoms are reported to lessen in some female patients during pregnancy and menstruation, although this phenomenon is not consistent, and the mechanism is not understood. Genetics
Most cases of EPP are results of inborn errors of metabolism but the metabolic defect in some patients may be acquired.
Bodies found at the scene of an accidental death often show evidence of other paraphilic activities, such as fetishistic cross-dressing and masochism. In cases involving teenagers at home, families may disturb the scene by "sanitizing" it, removing evidence of paraphilic activity. This can have the consequence of making the death appear to be a deliberate suicide, rather than an accident.The great majority of known erotic asphyxial deaths are male; among all known cases in Ontario and Alberta from 1974 to 1987, only 1 out of 117 cases was female. Some individual cases of women with erotic asphyxia have been reported. The main age of accidental death is mid-20s, but deaths have been reported in adolescents and in men in their 70s.Autoerotic asphyxiation has at times been incorrectly diagnosed as murder and especially so when a partner is present.Lawyers and insurance companies have brought cases to the attention of clinicians because some life insurance claims are payable in the event of accidental death, but not suicide. In fiction
The sensational nature of erotic asphyxiation often makes it the subject of urban legends. It has also been mentioned specifically in a number of works of fiction. In the Marquis de Sades famous novel Justine, or The Misfortunes of the Virtue, Justine is subjected to this by one of her captors. She survives the encounter. In the Guts short story in Chuck Palahniuks novel Haunted, one the characters discusses parents who discover the accidental deaths of their sons to autoerotic asphyxiation.