Lichen Sclerosus Flashcards
True or False
If left untreated, ~10% of patients with vulvar
LS may develop differentiated vulvar intraepithelial neoplasia (VIN) or squamous cell carcinoma (SCC)
False - 4%
Who does lichen sclerosus affect?
Occurs at all ages
No race prediliction
Thought to be female predominant
True or false
Lichen sclerosus often co-exists with morphea
True
What is the pathogenesis of morphea?
Genetic predisposition
- ass with MHC class II antigen HLA-DQ7
Immune dysregulation
- exact process unknown
- Increased production of IL 6, TNF and collagen
What are the associations of lichen Sclerosus?
1/ Morphea
2/ AI conditions (thyroid)
Describe the clinical features of Lichen Sclerosus
Most commonly affects the anogenital region
- can affect extragenital skin
Gential
- starts as single polygonal, bluish-white, shiny, slightly
elevated, interfollicular papules.
- Over time, these papules often enlarge or coalesce into larger plaques
- sclerotic, scar-like papules and plaques that are usually ivory in color and have a shiny and/or wrinkled surface
- Occasionally, lesions have a pink or light violet hue.
- in advanced stages: telangiectasias or follicular plugging, haemorrhagic bullae
- In women: figure-of-eight configuration, anatomical distortion
Cutaneous:
- dryness, occ pruritis
- favours the trunk and proximal extremities
Oral (rare);
- bluish-white papules up to 5 mm in diameter
may occur on the buccal mucosa or under the tongue
- scar-like atrophy or erosions
Complications of genital LS
For women:
1/ VIN or SCC in 4% of untreated cases
2/ Anatomical distortion
- dysparaenia, fissures, tears
- inability to have a vaginal delivery
For Men:
1/ phimosis
2/ recurrent balanitis
3/ SCC
Histopathology of lichen sclerosus?
Epidermis:
- thinned epidermis
- orthohyperkeratosis
- vacuolar degeneration of the basal layer.
- Hyperkeratosis is especially pronounced at follicular openings and may lead to plugging.
- flattening of the rete ridges
Dermis:
- superficial dermal edema
- band-like lymphocytic infiltrate
- homogenized dermal collagen
- loss of elastic fibres
- Subepidermal clefting and hemorrhage within the homogenized papillary dermis is frequently observed.
DDX of lichen sclerosus
Extragenital LS:
- Morphea
- GvHD
Genital LS:
- sexual abuse
- erosive lichen planus
- mucous membrane pemphigoid
- erythroplasia of Queyrat
- VIN
- SCC
Treatment of genital LS
Patient education
- Blitz treatment to control
- Maintenance therapy
General:
- frequent use of emoliants
- ointments better than creams
Topical:
- Topical corticosteroids
- Intralesional corticosteroids
- Topical calcinurin inhibitors
Phototherapy
- UVA1
- PDT
Surgery:
- circumscision
- fracitonal laser ablation
Treatment of secondary infection:
- swab and Rx
Treatment of extragenital LS
Topical CS
Topical vitamin D analouges
Phototherapy
- UVA1
Weekly methotrexate +/− systemic corticosteroids
Case report of dupilimab
