A 9-year-old female came to clinic with a 1 week history of perineal irritation and dysuria. This occurred mainly during the day, but it had awoken her from sleep. Her mother described the area as reddened and was not improving with sitz baths and emollients. She described that she didn’t have to urinate more frequently, nor did starting the urine stream bother her, but once the urine started flowing she had a pain. She had been afebrile and was otherwise feeling well. She denies any new soaps, detergents or other products including no new clothing. She wore cotton underwear and loose fitting clothing generally. She denied vaginal discharge and said that no one had touched her inappropriately in her genital area. She had no previous history of urinary tract infections and denied anal itching or pain. She did not have any significant dermatological or allergic history. There was no travel or animal contacts. The family history was positive for diabetes and maternal family members with thyroid problems.
The pertinent physical exam showed a well-appearing female with normal vital signs and growth parameters in the 10-50%. Her general skin examination showed no lesions. Her genital area revealed a pre-pubertal female with a pale white, shiny, continuous lesion involving the labia majora bilaterally that extended to the perineum. It did not extend to the anus and there was reddened skin lateral to the white area. It was well-demarcated but not “punched out” appearing. The affected area seemed thin and there were excoriations. There was no excessive vaginal discharge or odor. The hymen and other genital structures appeared normal.
The differential diagnosis of a urinary tract infection, post-irritation hypopigmentation such as due to pinworms, vitiligo or lichen sclerosus et atrophicus was considered. Vaginal foreign body or vulvovaginitis were considered but less likely given the physical examination. The attending pediatrician remarked to the resident, “This is a good example of why we rotate through all the specialties in medical school. We need to think about other things, but I think this is LS&A. I’ve seen it a couple of times in practice, but I learned about it in the adult gynecology clinic.” The resident pediatrician agreed that the patient’s lesion did appear similar to her experiences with adult women as well. “This isn’t the classic appearance but looks more like the top half of the hourglass,” the resident said. The laboratory evaluation of a urinalysis was normal. The patient was referred to the gynecology clinic who saw her the following day and confirmed the diagnosis. She was started on topical corticosteroids.
Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory disease with a strong autoimmune association. It can occur with other autoimmune diseases. Its specific cause is unknown. It affects females and males of all ages, but especially females 40-60 years. In the pediatric population it affects prepubertal females most often.
Treatment includes steroid medications or anti-inflammatory medications. Other treatments may be offered including phototherapy, and much less commonly systemic steroids or immunosuppressive therapy. Circumcision may also be an option for some males. Childhood LSA generally improves but can continue into adulthood. Scarring can occur with narrowing of the openings of the urethra, vagina, and anus and also the foreskin in males. Malignancy has been reported in adults but not children.
The differential diagnosis includes post-inflammatory hypopigmentation, lichen planus, tinea versicolor, vitiligo or morphea (localized sclerosis). Malignancies should also be considered in older populations. Vulvovaginitis and sexual abuse should be considered in the differential diagnosis for children because of the genital irritation presentation but usually do not have the same physical examination appearance.
LSA lesions are shiny, and pale or white. The lesion edges can appear more diffuse or well-demarcated. The tissue appears shiny, thin, wrinkled and parchment-like, while others will describe it as a “porcelain white appearing center surrounded by redness.”
The classic presentation of LSA are genital lesions that are described as hour-glass or a figure of 8 for females as it involves the vulva, perineum and anal areas. For males the most common involvement is of the foreskin which presents as phimosis. The glans and meatal involvement are also relatively common in males. Genital involvement tends to be diagnosed more quickly as it is commonly quite symptomatic with burning or pruritis. There can also be blistering or fissuring of the area. Constipation or dysuria due to pain from the fissuring area are common presentations as well. Extra genital involvement includes lateral thighs, buttocks, axilla and upper trunk including the breast. But can include other areas such as the face or orally. Extra-genital lesions tend to be diagnosed later as they are less symptomatic.
Questions for Further Discussion
1. What is in the differential diagnosis for vulvovaginitis? A review can be found here
2. What is the current antibiotic of choice for uncomplicated cystitis?
3. What are indications for referral to a gynecologist or urologist?
4. What are relative indications for medical circumcision?
- Age: School Ager
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Pope E, Laxer RM. Diagnosis and management of morphea and lichen sclerosus and atrophicus in children. Pediatr Clin North Am. 2014;61(2):309-319. doi:10.1016/j.pcl.2013.11.006
Tomo S, Santos I-S, de Queiroz S-A, Bernabe D-G, Simonato L-E, Miyahara G-I. Uncommon oral manifestation of lichen sclerosus: critical analysis of cases reported from 1957 to 2016. Med Oral Patol Oral Cir Bucal. 2017;22(4):e410-e416. doi:10.4317/medoral.21606
Akel R, Fuller C. Updates in lichen sclerosis: British Association of Dermatologists guidelines for the management of lichen sclerosus 2018. Br J Dermatol. 2018;178(4):823-824. doi:10.1111/bjd.16445
Green PA, Bethell GS, Wilkinson DJ, Kenny SE, Corbett HJ. Surgical management of genitourinary lichen sclerosus et atrophicus in boys in England: A 10-year review of practices and outcomes. J Pediatr Urol. 2019;15(1):45.e1-45.e5. doi:10.1016/j.jpurol.2018.02.027
Lichen Sclerosus. NORD (National Organization for Rare Disorders). Accessed May 18, 2021. https://rarediseases.org/rare-diseases/lichen-sclerosus/
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa