Lichen sclerosus Lichen planus Dr Fiona Lewis, Consultant Dermatologist, Heatherwood and Wexham Park NHS Foundation Trust & St John s Institute of Dermatology, GSTT
Outline Typical features of lichen sclerosus and lichen planus Diagnosis Management When to refer on
LS aetiology?? Genetic Infective Auto-immune
Skin immune system Increase in Langerhans cells at all stages of disease Increased ICAM1 and HLA-DR expression in treated disease Increased cytokine activity
What does NOT cause it? Sexually transmitted infection Hormone problems Allergy Diet Borelia burgdorferi HPV
Who gets LS? 35 30 25 20 15 Age of onset 10 5 0 16-19 20-29 30-39 40-49 50-59 60-69 70-79
Typical LS histology Thinned epidermis Homogenous band of collagen Lymphocytic inflammation
What are the symptoms? Pruritus Soreness Dyspareunia Constipation in children
What are the features? White plaques Ecchymosis Architectural change Fissures
How to manage a patient with lichen sclerosus
LS treatment what not to do! Arsenic Glycerol pomade of pyrogallol Radiotherapy Bismuth Mercury tattoos Testosterone Vulvectomy
How is it treated? Emollients Ultra-potent topical steroids (Cochrane reviews, BAD Guidelines 2018 etc)
PATIENT MANAGEMENT PATHWAY ADULT FEMALE ANOGENITAL LICHEN SCLEROSUS Please use in conjunction with the summary of recommendations and discussions in the guideline and supporting information British Association of Dermatologists MAKING A DIAGNOSIS Symptoms Itching, soreness, dysuria Superficial dyspareunia Clinical signs White plaques Ecchymosis Fissuring Architectural alteration Confident of clinical diagnosis and experienced in the management of LS? NO YES INITIAL TREATMENT (Primary or Secondary care) Clear documentation of clinical features using diagram or photograph Consider confirmatory biopsy Clobetasol propionate 0.05% ointment 3- month induction regimen (once daily for 1 month, alternate days for 1 month, twice weekly for 1 month). About half a fingertip unit with instructions regarding site application. A 30 g tube should be the maximum needed in 3 months. Soap substitute, emollients and avoid irritants Provide patient information (www.bad.org.uk/leaflets) TREATMENT FAILURE i.e. persistent symptoms and active disease, further scarring, fissures and hyperkeratosis despite treatment Check compliance Re-consider diagnosis; biopsy if needed Check for change of symptoms suggesting vulvodynia Check for urinary incontinence Continue topical steroid on regular regimen, e.g. once / twice weekly to maintain symptom control and prevent progressive scarring ASSESS RESPONSE AT 3 MONTHS Clinical assessment of symptoms and signs Check for appropriate topical steroid use Assess sexual function TREATMENT SUCCESS i.e. symptom control, no further alterations of architecture, good sexual function Topical steroid as needed for recurrent symptoms Continue with emollients DO NOT TREAT EMPIRICALLY WITH A POTENT STEROID Use 1% hydrocortisone with emollients Refer to a dermatologist or gynaecologist experienced in treatment of vulval disease (specialty may vary locally) REFER FOR SPECIALISED ADVICE Dermatology or Gynaecology, depending on local services: Excessive use of topical steroid Psychosexual issues If surgery is being considered, e.g. for recurrent fissuring of the posterior introitus, release of clitoral or labial adhesions FOLLOW UP AFTER A FURTHER 3-6 MONTHS Assess response as at 3 months Continue topical steroid as needed to control disease FOLLOW UP AFTER A FURTHER 6 MONTHS Assess response as at 3 months TREATMENT SUCCESS URGENT REFERRAL To Gynae-oncology via the 2-week wait pathway, if SCC is suspected at any stage FOLLOW UP EVERY 6-12 MONTHS Until treatment success achieved Topical steroid as needed Self-examination Explain signs for when to seek medical advice Provide patient information on discharge (www.bad.org.uk/leaflets) GP advice regarding re-referral criteria
Treatment regime Clobetasol propionate 0.05% ointment once a day for 1 month alternate days for 1 month twice a week for 1 month Then individualised to maintain control of symptoms and signs eg. once/week if needed
Safe amounts of topical steroids 30g in 3 months (adults) 30g in 6 months (children)
Lichen sclerosus Pallor often remains Scarring should be stopped Symptom control
Follow-up 3 months, 6 months, 12 months Discharge if well controlled with patient information Long term follow-up for any patient with atypical disease or history of VIN
LICHEN PLANUS
LP? Auto-immune aetiology 55-65% females Age of onset 6 th decade
Ano-genital LP Classic Erosive Vulvo-vaginal ginigival syndrome (DRB1*0201 allele Setterfield 1996) Hypertrophic
What are the symptoms of LP? May be asymptomatic Soreness Pruritus Dyspareunia Discharge
LP histology Saw-tooth epidermis Dense lymphocytic infiltrate Basal cell degneration
Hypertrophic LP Uncommon Often perianal?increased risk of SCC
LP - differential diagnosis Mucous membrane pemphigoid GVHD VIN
Look elsewhere! CLASSIC/HYPERTROPHIC Scalp Nails Oral cavity Perianal
Look elsewhere! EROSIVE LP Vagina Gingivae Lacrimal ducts External auditory meatus Oesophagus
LP - management Emollients Topical steroids as for LS Barriers for erosive disease (calcineurin inhibitors) Systemic agents Biologics, but potential side effects
Important considerations with LS and LP Auto-immune disease Scarring Malignancy Dysaesthesia
Association with auto-immune disease 40% circulating antibodies Thyroid disease, pernicious anaemia etc
Malignancy Taussig 1920 Wallace 1971 4% LS 73% in leukoplakia LP Leibowitch 1990 Zaki 1996 61% in SCC specimens 50% adjacent to SCC 3/61 Derrick 2000 3/22
Differentiated VIN Non HPV related Often found at edge of SCC associated with LS/LP
When to refer on LS/LP complicated by VIN Erosive LP multisystem disease LS/LP unreponsive to first line treatment
Summary Clinical features of LS and LP Basic management Look at other sites Specialised management is important Clinico-pathological correlation is vital