Patient Presentation
An 11-year-old healthy female came to clinic after having noticing a “bump” in her vaginal area after taking a shower. Her mother was uncertain what it was and so had made the appointment. She denied having any significant pain, itching, discharge or actual bleeding. She was premenarchal. She had normal bowel movements and urination. She denied any trauma to the area or potential sexual abuse. The past medical and family histories, and review of systems were non-contributory.
The pertinent physical exam showed a healthy female with normal vital signs who was tracking at the 75-90% for growth parameters. Her abdominal and spine exams were normal. Her genitourinary examination showed a small slightly elevated hematoma at the posterior fourcette. Areas of the labia major and minora looked to have thinned tissue and there was hypopigmentation that was linear around the edges of the labia minora and which extended along the center of the perineal body. There was some minor erythema of the areas around the hypopigmentation. There were other areas of hypopigmentation that were 3-5 mm in size on the labia majora and minora. The hymen was intact and the tissues and anatomy otherwise appeared normal.
The diagnosis of possible lichen sclerosis et atrophicus (LSA) was made. In retrospect, she said that she had had some pruritus in her genital area for the past few days and had been itching the area more. Pictures were taken for the medical record. The pediatrician checked the medical literature which recommended strong steroid treatment but possibly biopsy before treatment, so gynecology was consulted. They agreed that this was LSA and recommended steroid use without a biopsy. They would see the patient in about 3-4 weeks for followup.
Discussion
Lichen sclerosis et atrophicus (LSA) is a chronic inflammatory disease with a strong autoimmune association but its cause is unknown. Usually seen in middle-aged women (40-60 years), it can occur in females and males of all ages, but prepubertal females are more common in the pediatric age group. Treatment includes stronger steroid medications or anti-inflammatory medications and pediatric patients usually have resolution with time. The classic presentation of LSA are genital lesions that are hypopigmentation in an hour-glass or a figure of 8 distribution for females as it involves the vulva, perineum and anal areas. Tissues appear thinned and there can be some erythema as well. Patients can be asymptomatic, have some pruritus or pain. Extra genital lesions can also be seen. A review of LSA can be found here
Learning Point
The differential diagnosis of white vulvar lesions includes:
- Hypopigmentation
- Normal variant
- Post-inflammatory including general vulvovaginitis
- Vitiligo
- Angioedema
- Atopic dermatitis
- Psoriasis
- Seborrheic dermatitis
- Lichen sclerosis et atrophicus
- Lichen planus
- Lichen simplex chronicus
- Morphea or localized sclerosis
- Mycosis, ex. tinea versicolor
- Ulcer
- Lipschultz
- Herpes simplex
- Syphilis
- Other infections
- Scratching
- Tight clothing
- Burn
- Sexual assault
- Bechet’s
Scleroderma
- Basal cell carcinoma
- Squamous cell carcinoma
- Leukemia/lymphoma
Questions for Further Discussion
1. What is a Lipschultz ulcer? A review can be found here
2. How are straddle injuries treated? A review can be found here
3. What are some presentations for child sexual abuse? A review can be found here
Related Cases
- Disease: Lichen Sclerosis et Atrophicus | Vulvar Disorders | Skin Diseases
- Symptom/Presentation: Rash | Urticaria and Pruritis
- Specialty: Obstetrics / Gynecology | Dermatology
- Age: School Ager
To Learn More
To view pediatric review articles on this topic from the past year check PubMed.
Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.
Information prescriptions for patients can be found at MedlinePlus for these topics: Vulvar Disorders and Skin Conditions.
To view current news articles on this topic check Google News.
To view images related to this topic check Google Images.
To view videos related to this topic check YouTube Videos.
Resende FS, Conforti C, Giuffrida R, de Barros MH, Zalaudek I. Raised vulvar lesions: be aware! Dermatol Pract Concept. 2018;8(2):158-161. doi:10.5826/dpc.0802a16
Charamanta M, Soldatou A, Michala L. Vulvar Ulcers in Children: Dramatic But Self-Limited. Pediatric Emergency Care. 2021;37(2):70. doi:10.1097/PEC.0000000000002004
Orszulak D, Dulska A, Nizinski K, et.al. Pediatric Vulvar Lichen Sclerosus – A Review of the Literature. Int J. Environ. Res. Public Health. 2021:18;7153.
Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa
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