In ischemic priapism, the risk of permanent scarring of the penis begins to increase after four hours and definitely occurs after 48 hours. Priapism occurs in about 1 in 20,000 to 1 in 100,000 males per year. Classification
Priapism is classified into three groups: ischemic (low-flow), nonischemic (high-flow), and recurrent ischemic. The majority of cases (19 out of 20) are ischemic in nature.Some sources give a duration of four hours as a definition of priapism, but others give six. "The duration of a normal erection before it is classifiable as priapism is still controversial. Ongoing penile erections for more than 6 hours can be classified as priapism. "Priapism in females (continued, painful erection of the clitoris) is significantly rarer than priapism in men and is known as clitoral priapism or clitorism. It is associated with persistent genital arousal disorder (PGAD). Only a few case reports of women experiencing clitoral priapism exist. Signs and symptoms
Complications
Because ischemic priapism causes the blood to remain in the penis for unusually long periods of time, the blood becomes deprived of oxygen, which can cause damage to the penile tissue. Such damage can result in erectile dysfunction or disfigurement of the penis. In extreme cases, if the penis develops severe vascular disease, the priapism can result in penile gangrene. Low-flow priapism
Causes of low-flow priapism include sickle cell anemia (most common in children), leukemia, and other blood dyscrasias such as thalassemia and multiple myeloma, and the use of various drugs, as well as cancers.
As a consequence two strikingly similar cases were identified and a review of the literature revealed three more patients with similar characteristics and a novel multisystem disease, the TEMPI syndrome, was reported.As of January 2022, a total of 29 patients worldwide with the TEMPI syndrome have been identified. References
External links
Office of Rare Diseases Research (US)
Orpha.net (EU)
Hematopia TEMPI Page