Lichen Sclerosus; FGM; Congenital Abnormalities Flashcards
Define lichen sclerosus [1]
Lichen sclerosus is a chronic, inflammatory dermatosis of unknown aetiology that predominantly affects the anogenital region. It can occur in all age groups but is most common in postmenopausal women.
- It is associated with other autoimmune diseases, such as type 1 diabetes, alopecia, hypothyroid and vitiligo.
It is important not to get lichen sclerosus confused with lichen simplex or lichen planus.
What are these other conditions? [2]
Lichen simplex
- chronic inflammation and irritation caused by repeated scratching and rubbing of an area of skin. This presents with excoriations, plaques, scaling and thickened skin.
Lichen planus
- an autoimmune condition that causes localised chronic inflammation with shiny, purplish, flat-topped raised areas with white lines across the surface called Wickham’s striae.
Which parts of the body are affected by lichen sclerosus compared to lichen planus? [2]
Lichen sclerosus is normally confined to the vulva, typically does not affect the vagina and very rarely involves the oral mucosa, while lichen planus may affect the vagina, skin, hair, nails, as well as genital and oral mucous membranes.
Lichen sclerosus carries a small risk of malignant transformation to [] due to chronic inflammation and cellular atypia.
Lichen sclerosus carries a small risk of malignant transformation to squamous cell carcinoma due to chronic inflammation and cellular atypia.
Describe the presentation of anogenital lichen sclerosus [5]
Pruritus:
- This is typically the earliest and most common symptom. It may be severe and unresponsive to topical treatments.
Dyspareunia:
- Pain during sexual intercourse is a common complaint due to atrophic changes leading to skin fragility and fissures.
Pain and discomfort:
- These are frequently reported symptoms which may be exacerbated by secondary infection or trauma.
Physical changes: The skin may appear pale or white with a shiny surface. There may be visible thinning (atrophy) or thickening (hyperkeratosis).
Wrinkling of the skin may disappear (effacement), especially noticeable on the labia minora in women or foreskin in men.
Describe the anatomical alterations that can ocur in anogenital lichen sclerosus [3
Anatomic alterations: Chronic disease can lead to significant architectural distortion including phimosis in males, narrowing of the vaginal introitus, adhesions, fusion of labia minora, clitoral hood obliteration and burying of the clitoris in females.
Describe the extragenital lichen sclerosus manifestations [3]
This form typically presents as asymptomatic white patches with follicular delling (plugging). The plaques might have a smooth surface but hyperkeratotic papules could also be present.
* Most common sites of involvement are the trunk, proximal extremities and scalp. However, any part of the body can be affected excluding the palms and soles.
Which associated autoimmune pathologies are linked to lichen sclerosus? [4]
thyroid disease, vitiligo, alopecia areata and pernicious anaemia.
Describe the diagnosis of lichen sclerosus [2]
The RCOG advises the following:
- Skin biopsy is not necessary when a diagnosis can be made on clinical examination.
- Biopsy is required if the woman fails to respond to treatment or there is clinical suspicion of VIN or cancer
What are the 4 types of FGM? [4]
Type 1: Removal of part or all of the clitoris
Type 2: Removal of part or all of the clitoris and labia minora. The labia majora may also be removed.
Type 3: Narrowing or closing the vaginal orifice (infibulation).
Type 4: All other unnecessary procedures to the female genitalia.
You identify a case of FGM. How do you handle this situation?
It is mandatory to report all cases of FGM in patients under 18 to the police. Also get the following involved:
* Social services and safeguarding
* Paediatrics
* Specialist gynaecology or FGM services
* Counselling
In patients over 18:
- careful consideration about whether to report cases to the police or social services.
- The RCOG recommends using a risk assessment tool to tackle this issue (available on the gov.uk website). The risk assessment includes considering whether the patient has female relatives that may be at risk.
The upper vagina, cervix, uterus and fallopian tubes develop from the []
The upper vagina, cervix, uterus and fallopian tubes develop from the paramesonephric ducts (Mullerian ducts).
These are a pair of passageways along the outside of the urogenital region that fuse and mature to become the uterus, fallopian tubes, cervix and upper third of the vagina
NB: In a male fetus, anti-Mullerian hormone is produced, which suppresses the growth of the paramesonephric ducts, causing them to disappear.
Describe what is meant by a bicornuate uterus [1]
A bicornuate uterus is where there are two “horns” to the uterus, giving the uterus a heart-shaped appearance
- diagnosed on a pelvic ultrasound scan
- successful pregnancies are generally expected but typical complications include miscarriage, premature birth and malpresentation
Describe what is meant by an imperforate hymen [1]
Imperforate hymen is where the hymen at the entrance of the vagina is fully formed, without an opening.
Describe the presentation of an imperforate hymen [2]
What is the treatment? [1]
Cyclical pelvic pain and cramping that would ordinarily be associated with menstruation, but without any vaginal bleeding at the age of menarche
Treatment is with surgical incision to create an opening in the hymen.
Describe what is meant by a transverse vaginal space [1]
Transverse vaginal septae is caused by an error in development, where a septum (wall) forms transversely across the vagina.
This septum can either be perforate (with a hole) or imperforate (completely sealed).
- Where it is perforate, girls will still menstruate, but can have difficulty with intercourse or tampon use.
- Where it is imperforate, it will present similarly to an imperforate hymen with cyclical pelvic symptoms without menstruation
