Paediatric vulval lichen sclerosus: a retrospective study

Abstract

Summary Background Lichen sclerosus (LS) is a chronic inflammatory dermatosis with a predilection for the anogenital region, which mainly affects prepubertal girls and postmenopausal women. The cause is unknown, but a number of potential aetiological factors have been identified. Aim To examine a cohort of patients with prepubertal‐onset vulval LS (VLS) and assess baseline characteristics, clinical presentation, potential precipitating and predisposing factors, and response to treatment. Methods Data were collected from case notes on patients aged < 18 years diagnosed with prepubertal‐onset VLS attending a specialist vulval dermatology service. Data included clinical presentation, comorbidities, family history, therapy and response to treatment. Results In total, 26 paediatric patients were identified. The median age at onset of symptoms was 5 years (range 2–8.5 years). Many previously identified potential aetiological factors for the development of VLS were identified, including family history, trauma, autoimmune disease and hormonal factors. A significant proportion of patients had a history of urinary tract symptoms, including incontinence and urinary tract infection. Most patients responded well to a standard course of induction topical therapy followed by maintenance therapy, but some, including three patients with ongoing urinary incontinence and three postpubertal patients, continued to have active disease. Conclusion A detailed assessment is essential in patients with VLS so that potential predisposing factors and comorbidities can be identified and managed. Urinary incontinence may be implicated in the development of paediatric VLS and may prevent adequate disease control. Paediatric VLS can persist through puberty, thus long‐term follow‐up is advised.

Comparison of lichen sclerosus in boys and girls: A systematic literature review of epidemiology, symptoms, genetic background, risk factors, treatment, and prognosis

“…[23][24][25][26] In a later stage, anatomic changes can be seen, such as labial fusion. 27,28 Genital LS may be mistaken for sexual abuse. However, LS and sexual abuse are not mutually exclusive and, if suspected, abuse should be investigated.…”

“…Hypopigmentation, hyperpigmentation, erythema, fissures, atrophy, keratotic papules 19,22 Telangiectasias/purpura, angiokeratomas (rare) [23][24][25] Labial fusion 27,28 Hypopigmentation distal portion of the prepuce 35 Phimosis, scarring, secondary buried penis, balanitis Erythema, telangiectasias/purpura 11,35,36 Histopathology Hyperkeratosis (96%), basal vacuolization of keratinocytes (88%), lymphocytic exocytosis (91%), dermal sclerosis (99%) and epidermal atrophy (50%) 43 Hyperkeratosis (82.9%), hyalinization (100%), basal cell degeneration (56.1%), lymphocytic infiltration (100%), fibrin deposition (7.3%) 32 In 67% dermoepidermal clefts 44…”

Pediatric Vulvar Lichen Sclerosus—A Review of the Literature

“…Therefore, repeatable irritation is believed to be a possible factor contributing to the disease. Another possible causal factor may be radiation [ 18 , 19 , 20 ].…”

“…The clinical types of extragenital VLS include an extensive bullous form [ 19 , 21 ] and annular, blaschkoid and keratotic variants [ 22 ]. Koebnerization is very common at extragenital sites, arising at pressure points, old surgical and radiotherapy scars, and sites of trauma, including urostomies [ 18 ].…”

The SWIFT Model for Lichen Sclerosus Among Premenarchal Girls

“…The variables included in this model are consistent with signs common among other cohorts of premenarchal girls with LS. 1,3,9,[15][16][17][18][19] The SWIFT model accurately predicted LS in all patients except 5. Therefore, although the SWIFT model may be useful in predicting when a diagnosis of LS should be suspected, careful history taking and physical examination are crucial to determine whether a diagnosis of LS is appropriate within the patient setting.…”