Case 1: Because lichen sclerosis can look like a variety of lesions affecting the vulva clinically, biopsy is best performed. The edge of the lesion is the best place to biopsy. Extensive dermal sclerosis with minimal chronic inflammation is apparent. The epidermis shows loss of rete ridges and some attenuation, which overlies dermal homogenization.
Early vacuolar change is seen along the dermal-epidermal junction. Some pigmented macrophages are seen along with parakeratosis.
Case 2: This different case also shows vacuolar changes along the dermal-epidermal junction (arrow). A sparse chronic inflammatory infiltrate is present but not within the immediate subepithelial area.
Case 3: Again, note the pallor beneath the atrophic epidermis (superficial papillary pallor). This is due to edema with hyalinization of collagen. A rule of thumb in dermpath: if you see a lot of pink with no cells, it is termed sclerotic. If there is lots of pink with cells, it is fibrotic.
Some other features include follicular plugging by keratin.
Liquefaction degeneration (vacuolar change) along the dermal-epidermal junction and pigment incontinence are also common features in LS. Melanin is usually fine and granular, whereas hemosiderin is a bit chunkier.
Lichen sclerosus is a chronic skin disorder of skin and mucosal surfaces. It is most often encountered on the female genital area but may affect other areas of the body. Any age group may be affected, but it is more often in elderly women.
The exact cause of lichen sclerosus remains unknown. Interestingly, there are cases of family members with lichen sclerosus suggesting at least some cases may have a genetic link. There is also the possibility of autoimmune disorder.
Lichen sclerosus has a variety of appearances and is often associated with pruritis. Lesions may be focal or expansive, encompassing the entire vulva. Most commonly it appears as a thin crinkly whitened area. Fissuring and ulceration as well as a bruised appearance may also occur.
Ultra-potent topical corticosteroids are the first line treatment. A short-term burst of a high potency topical steroid is often followed by long-term use of a lower potency topical corticosteroid to prevent recurrence.
Other treatments include testosterone, progesterone, tacrolimus, surgery, and phototherapy. Surgery should be reserved for those symptomatic patients who fail multiple medical treatments, due to a high recurrence rate following surgery.
There is a 5% risk of developing squamous cell carcinoma of the vulva so adequate follow up and surveillance is important.
Smith YR, Haefner HK. Vulvar lichen sclerosus : pathophysiology and treatment. Am J Clin Dermatol. 2004;5(2):105-25.