Lichen Sclerosus
The condition you are describing is lichen sclerosus (LS), a chronic inflammatory dermatosis that causes loss of labial architecture through scarring, fusion of the labia minora, and can lead to fissures and ulcerations. 1
Clinical Presentation
Lichen sclerosus is a scarring process that causes loss of the labia minora, sealing of the clitoral hood, and burying of the clitoris. 1 The disease presents with:
- Porcelain-white papules and plaques affecting the interlabial sulci, labia minora, clitoral hood, clitoris, perineal body and perineum 1
- Fragile, thinned and atrophic skin with a characteristic figure-eight pattern around the vulva and anus 1
- Fissures and tears that develop with time, leading to pain and potential ulceration 1
- Scarring that causes fusion of the labia minora and narrowing of the introitus 1
- Areas of ecchymosis (bruising) often accompanying the white plaques 1
Key Diagnostic Features
The loss of labial distinction occurs through progressive scarring:
- Fusion of the labia minora eliminates the normal anatomical boundaries between structures 1
- Obliteration of the labia minora can occur in advanced disease 2
- Stenosis of the introitus develops as scarring progresses 2
- Buried clitoris results from sealing of the clitoral hood 1
Ulceration Mechanism
Fissures and ulcerations develop because the involved skin becomes inelastic and prone to tearing. 1 The fragile, atrophic skin is susceptible to:
- Mechanical trauma during daily activities 1
- Erosions that cause pain 1
- Fissuring that can be severe enough to cause dyspareunia and pain with defecation 1
Epidemiology
Lichen sclerosus has a bimodal age distribution with peaks in prepubertal girls and postmenopausal women. 1 The disease:
- Affects the anogenital area in 85-98% of female patients 1
- Is more prevalent in women than men with ratios of 6:1 to 10:1 1
- Most commonly presents in postmenopausal women in the fifth to sixth decade of life 1
Important Clinical Pitfall
In prepubertal girls, the ecchymosis and fissuring of lichen sclerosus may be mistaken for signs of sexual abuse. 1 However, the diagnosis of lichen sclerosus does not exclude the possibility of concurrent abuse, as trauma may trigger disease onset through Koebnerization 1.
Associated Symptoms
Beyond the structural changes:
- Intractable pruritus is the main symptom, often worse at night 1
- Dyspareunia occurs with erosions, fissures, or introital narrowing 1
- Dysuria and pain on defecation from perianal involvement 1
- Some patients remain asymptomatic despite visible disease 1
Etiology
Lichen sclerosus is considered an autoimmune inflammatory dermatosis with evidence including: