Vulval disease in children

Transcription

1 Vulval disease in children SYDNEY MEDICAL SCHOOL NORTHERN Associate Professor Gayle Fischer MBBS MD FACD I have no conflict of interests

2 Range of vulval diseases in children Inflammatory dermatoses Infections Allergic reactions Blisters and ulcers Naevi and Infiltrates Psoriasis S.Pyogenes Vulvovaginitis Allergic contact dermatitis Aphthae Haemangioma of infancy Atopic dermatitis HPV Fixed Drug Eruption Lichen Sclerosus Molluscum contagiosum Bullous pemphigoid Erythema multiforme Epidermal naevi Pigmented naevi Contact Dermatitis S.aureus Syringoma Labial fusion Tinea JXG Crohn s Mastocytoma

3 Whom do we consider a child? The literature considers children to be <18 Vulval disease in pre-pubertal children is distinct medically Vulval disease in adolescents is distinct emotionally, not medically 3

4 Female adolescent development 4

5 What does the literature say about paediatric vulval conditions? There is very little information in the medical literature. A recent review (2017) stated Vulvovaginitis in girls is caused by nonspecific factors and hygiene measures and recommended bio-yoghurt, probiotics, nanotechnology and petroleum jelly. Another review (2016) stated Our findings suggest that vulvovaginitis in prepubertal girls is related not only to micro-organisms, but also poor personal hygiene, the educational status of mothers, and specific irritants. 5

6 Common parental experiences Child abuse fears My child is abnormal Issues at school Embarrassment Frustration with diagnoses such as thrush, worms, UTI Fear regarding use of topical corticosteroids

7 Examining children with vulval disease Children remember these encounters: keep them positive. Small girls: mum s lap Let the child decide when it is time to get on the bed Never force an examination Photos can help Don t touch without permission Manage parents fear and expectations 7

8 Genital Dermatitis Common cause of genital itch and irritation Signs can be subtle Atopic children Exposed to irritants Rarely acute contact dermatitis

9 Dermatitis Treatment 1% hydrocortisone ointment Emollient Rule out and treat superinfection: usually S.aureus Reassurance and explanation

10 Irritants and practical advice for parents Night nappies Swimming lessons Bubble bath and shampoo Ballet lessons Cycling and horse-riding Fecal incontinence and dysfunctional voiding Stop using antifungals and wet wipes (MI) It is VERY unusual to have a hygiene problem

11 Less common than dermatitis generally More common as a cause of genital rashes Vulval Psoriasis in children

12 Causes of Non-Specific Vulvitis (Fischer G Australas J Dermatol 2010;; 51:118) Pre- pubertal n=38 Post- menarchal n=68 Dermatitis 9 (24%) 14 (21%) Psoriasis 27 (71%) 8 (12%) Chronic vulvovaginal candidiasis 0 42 (62%) p<0.001

13 Genital psoriasis: Treatment Same environmental modification as for dermatitis Stronger topical corticosteroids Steroid sparer : emollient, tar ointment

14 Lichen Sclerosus Mean age at onset 5-7 years Commonest presenting symptoms: itch and soreness Other symptoms at presentation - Dysuria - Constipation Referrals to urology and gastro-enterology common May be mistaken for sexual abuse

15 Management of LS in Girls No different to adult women Disease suppression with potent topical corticosteroid: well validated Long-term control vital with pre-pubertal onset disease to prevent scarring Often a delay in diagnosis 50% have scarring at presentation Genital lichen sclerosus in childhood and adolescence-a retrospective case series of 15 patients: early diagnosis is crucial to avoid long-term sequelae. Nerantzoulis I;; Grigoriadis T;; Michala L. European Journal of Pediatrics. 176(10): , 2017 Oct. Ellis E, Fischer G. Pre-pubertal onset vulvar lichen sclerosus: The importance of maintenance therapy in long-term outcomes. Pediatr Dermatol 2015 Treatment of vulvar lichen sclerosus with topical corticosteroids in children: a study of 72 children.casey GA;; Cooper SM;; Powell JJ. Clinical & Experimental Dermatology. 40(3):289-92, 2015 Apr.

16 Prognosis Vulvar lichen sclerosus does not resolve at puberty When still present at puberty remission unlikely Early onset malignancy in adult life Inadequately treated childhood disease may result in scarring Powell J, and Wojnarowska F. Childhood vulvar lichen sclerosus: the course after puberty. J Reprod Med. 2002;;47: Smith S and Fischer G. Childhood onset vulvar lichen sclerosus does not resolve at puberty: A prospective case series. Pediatr Dermatol 2009;; 26: The course of lichen sclerosus diagnosed prior to puberty.focseneanu MA;; Gupta M;; Squires KC;; Bayliss SJ;; Berk D;; Merritt DF. Journal of Pediatric & Adolescent Gynecology. 26(3):153-5, 2013 Jun.

17 Management of LS in children Outcome Adherent Non-adherent P-value Time to initial remission 4.2 months 5.2 months Complete control on maintenance treatment 91% 8% <0.001 Progression of disease during treatment 0 69% <0.001 Progression of scarring 0 16% <0.001 Side effects (erythema, irritation) 4 (12%) 2 (15%) >0.99 Ellis E, Fischer G. Pre-pubertal onset vulvar lichen sclerosus: The importance of maintenance therapy in long-term outcomes. Pediatr Dermatol 2015

18 Implications for practice Maintenance treatment can prevent progression of disease Opportunity for early intervention Potentially reduce future cancer risk Regular preventative rather than as needed treatment

19 Streptococcal Genital Infection Presentation: acute, sore, red May be associated perianal infection Source: oropharynx May recur due to pharyngeal carrier state Swabs: Group A Beta-Hemolytic Strep May precipitate psoriasis Treatment: appropriate antibiotic 10 days

20 Does thrush happen to pre-pubertal children? Studies that EXCLUDE teenagers in the cohort have found that C.albicans is rarely found in genital isolates C.albicans clinical infection requires oestrogenisation Unless Recent oral antibiotics Diabetes Immunosuppression

21 Microbiology: (Fischer G Australas J Dermatol 2010;; 51:118) Pre- pubertal n=38 Post- menarchal n=68 C.albicans(causal) 0 35 (50%) p=<0.001 C.Albicans (colonization) 2 (5%) 3 (4%) Gp A β- haem strep 4 (10%) 0 H.Influenzae 3 (7.5%) 0 S. Aureus 1 (2.5%) 2 (3%) Gardnerella 0 1 (1.5%) No pathogen isolated 28(74%) 28(41%)

22 Aphthous ulceration There is a plethora of names for this Benign, non-infective acute and/or recurrent ulceration Involves mucosal and adjacent surfaces only Painful Recurrent in 50%

23 Acute Aphthosis Early adolescent to teenage girls Ulceration of sudden onset Flu-like prodrome in most Ulcers often large(>10mm) May be obscured by intense swelling of labia Incapacitating Patients often unable to walk or urinate

24 All forms of aphthae are frequently misdiagnosed: - HSV - Other forms of STI - Trauma First do no harm Problem of diagnosis For young girls this can present a major source of iatrogenic trauma

25 Aetiology of Aphthae Acute form may be reactive, virally induced EBV has been implicated but not always found Recurrent form: many theories, essentially unknown Helicobacter, Mycoplasma, other micro-organisms Oral aphthae associated with Coeliac Disease Aphthous-like ulcers seen in Crohn s, Behçet s and PFAPA syndrome

26 Management of Aphthae No evidence-based treatment guidelines exist The literature is vague on management There are recommendations for treating oral aphthae which can be extrapolated

27 Investigations should be minimal: Viral swab Serology interesting but does not aid management Severe: oral prednisone 0.5-1mg/kg daily Mild: potent TCS Analgesia Wait.. reassure If recurs, prophylaxis: Doxycycline mg/day Colchicine may be used Non-invasive approach to young girls Management of Aphthae Recommended management Dixit S, Bradford J, Fischer G. Management of nonsexually acquired genital ulceration using oral and topical corticosteroid followed by doxycycline prophylaxis.j Am Acad Dermatol /j.jaad

28 Vaginal foreign bodies: a common problem? No Worth considering if persistent discharge Said to be toilet paper most often In practice, rare Treat with saline lavage

29 Recurrent toxin-mediated perineal erythema Recurrent asymptomatic erythema and desquamation of vulva and perineaum Follows a Streptococcal pharyngitis Superantigen phenomenon May be desquamation of hands, feet Associated with strawberry tongue DD: Kawasaki Syndrome Rx: Penicillin

30 Staphylococcal Folliculitis Often also folliculitis on buttocks Itchy rather than sore, pustules S. Aureus is cultured Consider perineal staph carriage May superimpose on any skin condition

31 Genital HPV Not always the result of sexual transmission but this needs consideration in all cases. Remains controversial We don t know outcome re later cervical infection If maternal source: screen mother Treatment: imiquimod, podophyllin Observation only

32 Molluscum Contagiosum Common viral infection in children Spread in water Uncommon as isolated vulval presentation Not usually considered an STD in children. 4 genotypes: 2 are sexually acquired May be giant May be extensive in children with HIV

33 Tinea of the vulva in children Very rare Often incognito due to treatment Diagnosis: scraping Rx: Topical antifungal

34 Naevi and other lesions Haemangiomas Pigmented naevi Epidermal naevi Hamartomas

35 Birthmarks Birthmarks are commonly seen on the vulva in children. Any birthmark that occurs on skin generally may be found on the vulva They are often confused with dermatoses and genital warts Haemangionas may be confused with sexual abuse

36 Hidradenitis Suppurativa in Children HS has been reported in children as young as 5 More often a family history Look for androgenisation Most common is premature adrenarche Treatment: spironolactone, antibiotic combinations 36

37 Blistering Disease Vulval bullous pemphigoid Linear IgA disease Erythema Multiforme Fixed Drug Eruption Vesicular nappy rash Allergic contact dermatitis Erosive lichen planus is very rare in children (more likely to be BP)

38 Childhood vulval bullous pemphigoid Erosive or blistering disease Painful May be cicatricial May be associated with ocular disease Biopsy with IF diagnostic Responds to potent topical TCS

39 Pseudoverrucous papules and nodules (PPN) Variant on irritant contact dermatitis of the genital skin Alarming appearance Papules, nodules Vesicles Often secondary to persistent, severe maceration Keep dry, remove irritants Dixit S, Scurry J, Fischer G. A vesicular variant of pseudoverrucous papules and nodules in the genital area of an incontinent 4 year old. Australas J Dermatol

40 Vulval fixed drug eruption Occurs in children Acute erosive vulvitis Ibuprofen Paracetamol Sulfa antibiotics Often missed Can scar

41 Labial Fusion Seen only in young children often with an underlying vulvitis Not seen in adults Abnormal vulval appearance Confused with ambiguous genitalia Asymptomatic, but pooling of urine may result in vulvitis or urethritis Treated with topical oestrogen cream and potent topical corticosteroids Often recurs after treatment until puberty Clinical Recommendation: Labial Adhesions.Bacon JL;; Romano ME;; Quint EH. Journal of Pediatric & Adolescent Gynecology. 28(5):405-9, 2015 Oct. How should we treat prepubertal labial adhesions? Retrospective comparison of topical treatments: estrogen only, betamethasone only, and combination estrogen and betamethasone.eroglu E;; Yip M;; Oktar T;; Kayiran SM;; Mocan H. Journal of Pediatric & Adolescent Gynecology. 24(6):389-91, 2011 Dec.

42 FGM, Labioplasty and adolescents FGM: the partial or total removal of the female external genitalia or other injury to the female genital organs for cultural, religious,or nontherapeutic reasons. Adolescents are increasingly asking for reduction of labia minora Asymmetrical labia minora can occur transiently in adolescence Surgery: not until adult life, fully developed Cosmetic Labiaplasty in an Adolescent Population. [Review]Runacres SA; Wood PL. Journal of Pediatric & Adolescent Gynecology. 29(3):218-22, 2016 Jun. Protruding labia minora: abnormal or just uncool?.michala L;; Koliantzaki S;; Antsaklis A. Journal of Psychosomatic Obstetrics & Gynecology. 32(3):154-6, 2011 Sep 42

43 Study of 1491 patients 1491 Patients Genital signs of child abuse, sexual and non-sexual 20% of women report some form of sexual abuse in childhood Diagnostic findings in 7% (99) Findings more likely if child has been penetrated However many children who are abused are not penetrated Primary predictor of diagnostic finding is history of abuse from the child: this is rare Listen for the word inside. Even experienced child protection doctors find signs difficult to interpret Doctors are mandatory reporters 43

44 Vulval pain in children and adolescents (Vulvodynia) Neuropathic vulval pain has been described Look for scoliosis Attention getting behavior in children Anxiety and depression in adolescents Dyspareunia and tampon intolerance in adolescents. Vulvodynia in adolescence: childhood vulvar pain syndromes. [Review] Clare CA; Yeh J. Journal of Pediatric & Adolescent Gynecology. 24(3):110-5, 2011 Jun 44

45 Key point: Genital dermatoses Chronic genital disease in children is uncommon relative to adults Almost all with genital itch and redness have psoriasis or dermatitis Children with genital dermatoses require TCS, not antifungals

46 Key Points: Lichen Sclerosus LS in children should be treated aggressively with potent TCS LS does not resolve at puberty LS may result in scarring if inadequately treated Follow up is essential to ensure adherence to long-term treatment

47 Key Points: Genital Infections The commonest genital infective condition in children is due to Gp A Streptococcus, which is not seen in adults Children do not suffer from recurrent or chronic candidiasis

48 Key Points: Genital ulceration Acute genital ulceration in adolescent girls is usually not infective Minimize investigations: HPV only Acute treatment with prednisone and analgesia Rarely recurs Recurrent disease: doxycycline

49 Summary: Key Points The commonest chronic genital dermatosis is psoriasis Lichen sclerosus requires long-term treatment and follow up Genital candidiasis does not occur in healthy pre-pubertal children Non-sexually acquired acute genital ulceration is an important differential from STI and child sexual abuse