Peer Reviewed


A Rare Case of Exclusively Extragenital Lichen Sclerosus in a Child

Patricia Zhao, BS1 • Nicole Edmonds, BA1 • R. Hal Flowers, MD2

1Medical Student, Department of Dermatology, University of Virginia School of Medicine, Charlottesville, VA
2Assistant Professor, Department of Dermatology, University of Virginia School of Medicine Charlottesville, VA

Zhao P, Edmonds N, Flowers RH. A rare case of exclusively extragenital lichen sclerosus in a child. Consultant. 2022;62(10):e30-32. doi:10.25270/con.2022.07.00005

Received January 4, 2022. Accepted February 10, 2022. Published online July 7, 2022.

The authors report no relevant financial relationships.

The authors report no relevant disclaimers.

Patricia Zhao, 1221 Lee Street, PO Box 800718, Charlottesville, VA 22903 (



Lichen sclerosus (LS) is a chronic, inflammatory skin condition of unknown etiology that shows a predilection for the anogenital region. Although LS primarily affects postmenopausal women, some cases do occur in children. However, in the pediatric setting, LS almost always affects the anogenital region with exclusively extragenital involvement very rarely diagnosed in childhood. Here, we present a 6-year-old girl with a 2-year history of thin, pink macules and plaques on her chest and back, which biopsy revealed to be extragenital LS. A review of this case and the literature describes the epidemiology, clinical features, pathophysiology, and treatment options for pediatric extragenital LS.

Key words: Lichen sclerosus, pediatric dermatology, skin biopsy, dermatopathology



Lichen sclerosus (LS) is a poorly understood, chronic, inflammatory skin condition that primarily affects the anogenital region.1,2 While LS has been reported in all age groups and both sexes, it primarily affects women in the fifth to sixth decades of life with only 7% to 15% of cases occurring in children.1,3  In the pediatric setting, LS almost always affects the anogenital region with exclusive extragenital involvement very rarely diagnosed in childhood.4 Here, we preset a case of extragenital LS without anogenital involvement in a pediatric patient in order to raise awareness of this rare disease subtype.

Case Presentation

A 6-year-old white girl with a history of mild eczema presented to the dermatology clinic because of a 2-year history of a nonpruritic, nonpainful rash on her back.

History. The patient had a family history of allergies and asthma in both her mother and father but no other family history of skin conditions, nail abnormalities, or autoimmune disease. She had previously been treated with topical hydrocortisone and over-the-counter antifungal cream without relief. The rash did resolve with use of triamcinolone 0.1% ointment but recurred whenever use of the ointment was discontinued.

Physical examination. Physical examination was notable for very thin, pink to whitish macules to minute plaques with micaceous overlying scale and slight atrophy on the patient’s chest and back (Figure 1). A solitary pit was also observed on the right thumbnail. The patient’s scalp, ears, genitals, and perianal area did not display signs of rash.

Differential diagnosis. The initial differential diagnosis included extragenital lichen sclerosis (LS), atrophic lichen planus, unusual psoriasis, and pityriasis lichenoides chronica. Discoid lupus erythematous and Degos disease were also considered, although these were lower on the differential.


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Figure 1. Thin, pink to whitish macules to minute plaques with micaceous overlying scale and slight atrophy scattered along the patient’s (A) back and (B) chest.


Diagnostic studies. Because of the uncertainty of the diagnosis, a punch biopsy was performed on one of the lesions on the right upper back. The biopsy revealed a slightly atrophic epidermis with vacuolar basal keratinocytes and rare dyskeratosis (Figure 2). The superficial dermis was notable for a hyalinized stroma and band-like lymphocytic infiltrate below the fibrosis, prototypical features seen in LS (Figure 2, panels B and C). There was no increase of dermal mucin, periadnexal lymphocytic infiltrate, or basement membrane thickening seen with hematoxylin and eosin staining. The histopathology in conjunction with the physical examination findings confirmed a diagnosis of extragenital LS.


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