Lichen sclerosus. Lichen planus

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1 Lichen sclerosus Lichen planus Dr Fiona Lewis, Consultant Dermatologist, Heatherwood and Wexham Park NHS Foundation Trust & St John s Institute of Dermatology, GSTT

2 Outline Typical features of lichen sclerosus and lichen planus Diagnosis Management When to refer on

3 LS aetiology?? Genetic Infective Auto-immune

4 Skin immune system Increase in Langerhans cells at all stages of disease Increased ICAM1 and HLA-DR expression in treated disease Increased cytokine activity

5 What does NOT cause it? Sexually transmitted infection Hormone problems Allergy Diet Borelia burgdorferi HPV

6 Who gets LS? Age of onset

7 Typical LS histology Thinned epidermis Homogenous band of collagen Lymphocytic inflammation

8 What are the symptoms? Pruritus Soreness Dyspareunia Constipation in children

9 What are the features? White plaques Ecchymosis Architectural change Fissures

10 How to manage a patient with lichen sclerosus

11 LS treatment what not to do! Arsenic Glycerol pomade of pyrogallol Radiotherapy Bismuth Mercury tattoos Testosterone Vulvectomy

12 How is it treated? Emollients Ultra-potent topical steroids (Cochrane reviews, BAD Guidelines 2018 etc)

13 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 ( 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 ( GP advice regarding re-referral criteria

14 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

15 Safe amounts of topical steroids 30g in 3 months (adults) 30g in 6 months (children)

16 Lichen sclerosus Pallor often remains Scarring should be stopped Symptom control

17 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

18 LICHEN PLANUS

19 LP? Auto-immune aetiology 55-65% females Age of onset 6 th decade

20 Ano-genital LP Classic Erosive Vulvo-vaginal ginigival syndrome (DRB1*0201 allele Setterfield 1996) Hypertrophic

21 What are the symptoms of LP? May be asymptomatic Soreness Pruritus Dyspareunia Discharge

22 LP histology Saw-tooth epidermis Dense lymphocytic infiltrate Basal cell degneration

23 Hypertrophic LP Uncommon Often perianal?increased risk of SCC

24 LP - differential diagnosis Mucous membrane pemphigoid GVHD VIN

25 Look elsewhere! CLASSIC/HYPERTROPHIC Scalp Nails Oral cavity Perianal

26 Look elsewhere! EROSIVE LP Vagina Gingivae Lacrimal ducts External auditory meatus Oesophagus

27 LP - management Emollients Topical steroids as for LS Barriers for erosive disease (calcineurin inhibitors) Systemic agents Biologics, but potential side effects

28 Important considerations with LS and LP Auto-immune disease Scarring Malignancy Dysaesthesia

29 Association with auto-immune disease 40% circulating antibodies Thyroid disease, pernicious anaemia etc

30 Malignancy Taussig 1920 Wallace % LS 73% in leukoplakia LP Leibowitch 1990 Zaki % in SCC specimens 50% adjacent to SCC 3/61 Derrick /22

31 Differentiated VIN Non HPV related Often found at edge of SCC associated with LS/LP

32 When to refer on LS/LP complicated by VIN Erosive LP multisystem disease LS/LP unreponsive to first line treatment

33 Summary Clinical features of LS and LP Basic management Look at other sites Specialised management is important Clinico-pathological correlation is vital