Intersex Variations Glossary
This glossary was created to provide people-centered, educational definitions about a wide variety of intersex variations and how they can manifest in people’s bodies. Intersex is still often referred to as “differences of sex development” (DSD) by the medical community. [read interACT’s Statement on Intersex Terminology]
What is the definition of intersex?
Intersex is an umbrella term for unique variations in reproductive or sex anatomy. Variations may appear in a person’s chromosomes, genitals, or internal organs like testes or ovaries. Some intersex traits are identified at birth, while others may not be discovered until puberty or later in life.
People with intersex traits have always existed, but there is more awareness now about the diversity of human bodies. People with intersex bodies sometimes face discrimination, including in healthcare settings, as early as infancy. There are over 30 medical terms for specific combinations of intersex traits. Every intersex person is different.
Sex characteristics is a term that often refers to the internal and external traits of an individual’s body. Gender and sexual orientation are different concepts. Intersex people can have any gender identity and sexual orientation.
Potential causes of intersex traits include random genetic variations, changes in a person’s number of sex chromosomes, gonadal differences, natal exposure to unusual levels of sex hormones, or different responses to sex hormones. Intersex traits in and of themselves are not life-threatening, although they are sometimes associated with other serious medical symptoms, such as with salt-wasting congenital adrenal hyperplasia (SW CAH) and turner syndrome.
Why make this glossary?
While there is information across the internet about these intersex variations, much of this information is:
- Pathologizing, reducing people to “defects;”
- Difficult to understand, written in medical journals or studies;
- Gendered, in a way that may or may not align with each person’s gender identity;
- Not representative of the full scope of how variations can look.
It also can be difficult to find lists of intersex variations gathered in one place, and our hope in gathering them together here is to both raise awareness of intersex variations and counter the isolation people might otherwise feel by showing the wide range of traits that exist under the intersex umbrella.
What this glossary is NOT:
- Fully comprehensive—we are discovering new intersex variations every day.
- A tool to diagnose yourself—we are not doctors, and the information in this resource is not medical advice.
- A definitive guide of what is intersex and what is not intersex—people may disagree about whether certain variations should “count” as intersex, although interACT uses the terms “variations in sex characteristics” and “intersex variations” as synonyms.
Why include the variations we included?
This glossary takes the perspective that intersex is a broad category, including both traits classically thought of as intersex and those that may not be. In this resource, we aim to include definitions for any innate physical trait that falls under the umbrella of variations in sex characteristics, generally meaning that the variation:
- Shows up in a person’s chromosomes, genitals, gonads or other internal reproductive organs, or how their body produces or responds to hormones;
- Differs from what society or medicine considers to be “typical” or “standard” for the development, appearance, or function of female bodies or male bodies; and
- Is present from birth or develops spontaneously later in life.
Some examples of things that would not be considered variations in sex characteristics under these guidelines would be:
- Having an uncircumcised penis (since it is typical to be born with foreskin)
- Experiencing changes related to a typical menopause
- Any alterations to sex characteristics caused by a medical procedure or injury.
Intersex is a spectrum, as is each variation. Not everyone represented by this list will feel that they fall under the intersex umbrella—although we encourage everyone here for whom the term resonates to use it. If you are only now discovering you might be intersex, we encourage you to get in contact with our interACT Youth Group (for those under 29) or InterConnect Support Group (for anyone!). We hope that anyone represented in this document, whether or not they identify as intersex, finds the information useful.
A Note on “Typical”
When we are talking about variations in sex characteristics, we are by definition talking about traits that may not be considered “typical” since they do not align with the sexrelated stereotypes created by medicine and society.
For clarity, we will use the words “typical” and “typically” in this glossary to mean that a characteristic, combination of characteristics, or a process of development is considered typical based on societal or medical standards.
We will use words like “often” or “generally” when describing the ways that traits tend to show up for a group of people with a specific variation.
No one’s value depends on whether or not their body conforms to sex or gender stereotypes. There is no one normal or “right” way to be a man, a woman, or a person outside of the gender binary.
Variations
5-alpha reductase deficiency (5-ARD)
17-beta Hydroxysteroid Dehydrogenase 3 Deficiency (17-beta)
Androgen Insensitivity Syndrome (AIS)
Anorchia
Aphallia
Aromatase Deficiency
Aromatase Excess Syndrome (Hyperestrogenism)
Bladder Exstrophy
Clitoromegaly
Cloacal Exstrophy
Chimerism
Complete Androgen Insensitivity Syndrome (CAIS)
Congenital Adrenal Hyperplasia (CAH)
Congenital chordee
Cryptorchidism
De la Chapelle Syndrome
Disorders of Sex Development, Differences in Sex Development, or DSD
Epispadias
Estrogen Insensitivity Syndrome (EIS)
Follicle-Stimulating Hormone (FSH) Insensitivity
Fraser Syndrome
Goldberg-Maxwell Syndrome (this is another name for Androgen Insensitivity Syndrome)
Gonadal dysgenesis