Lichen Sclerosus of Face: A Case Report and Review of Literature.

We present a case of lichen sclerosus (LS) involving face and review of the relevant literature since 1970. This article highlights the rarely reported manifestation of LS. Early diagnosis with dermoscopy or confocal microscopy and early treatment might help cure it, and it may have less malignant potential.

What was known?

Lichen sclerosus (LS) is a chronic inflammatory dermatosis that is characterized by the involvement of vulval and perianal region. Extragenital LS has been considered uncommon; however, the involvement of face is extremely rare.

Introduction

Lichen sclerosus (LS) is a chronic inflammatory disease that primarily causes vulval and perianal lesion in prepubertal, peri- and post-menopausal women, and the presence of white papules and atrophic patches. It was first described by Hallopeau in 1887. The pathogenesis is unknown, but it seems to be related to genetic susceptibility and autoimmune mechanisms.[1] LS is more common in women than men, and the ratio of female to male ranging from 6:1 to 10:1.[2] Extragenital LS has been considered uncommon, seen in about 15–20% patients of LS.[1] It is most common on the neck, shoulders, axillae, trunk, buttocks, thighs, and wrists.[3] However, the involvement of face is extremely rare.

Case Report

A 25-year-old girl, without any history of trauma or any other significant medical history or any family medical history, presented with white patch over her nose for 3 years. It started as erythema, expanding gradually, and finally turned out to be a white patch without any symptom. Examination revealed a well-demarcated, mild atrophic porcelain-white patch with a violaceous periphery over the left side of her nose. Close inspection of the affected area revealed the presence of the comedo-like openings on the surface of the lesion [Figure 1a]. There were no other sites involved, and systemic examination was normal. Histopathologic examination of the lesion showed atrophy of the epidermis, follicular plugging, focal basal cell vacuolization, and homogenization of dermal collagen along with a moderate lymphomononuclear cell infiltrate [Figure 1b and c]. Hemogram, serum biochemistry, and other examinations were normal. All of these findings were consistent with the diagnosis of LS. After the diagnosis was made, oral triamcinolone was prescribed with 8 mg/day combined with topical 0.1% tacrolimus twice a day, the lesion does not expand anymore with partial remission.

Figure 1

(a) The lesion on the nose (b and c) pathological feature of LS (H and E, ×100)

Discussion

We reviewed relevant literature of LS involving face, yielding 6 cases since 1970.[456789] The seventh case is reported in this review. Histologic features of biopsy in each case confirmed the diagnosis of LS. According to the review [Table 1], we found that as the lesion of vulvar is always itching, LS of face is always asymptomatic and appears as ivory- or porcelain-white macules, papules, and plaques. It prefers to affect the infraorbital (3/7), forehead (2/7), and nose (2/7). Three of nine follow the lines of Blaschko. Sometimes, it can expand, which is a sign of beginning aggressive treatments. The age of onset is also around prepubertal, perimenopausal, and postmenopausal, which is correspondence with LS. The sex ratio is almost equal (female to male is 4:3), which is a little different from LS of other sites. Interestingly, almost all the cases affected the right side except ours’, so it still needs more cases to prove and further exploration. Treatment may be effective with oral or intralesional steroids, and topical use of steroids can also lead to remission; however, topical use of tacrolimus only may have minimal benefit. It is not easy to cure it all. No case shows LS involving face result in cutaneous tumors.

Table 1

Summary of reported cases of lichen sclerosus of face

Though LS of face is rare on the face, it still need us pay attention to this rarely reported manifestation of LS. Attili and Attili[10] reviewed the lip LS, which reminds us that it can occur in any sites. Atypical locations would be the palmar and plantar regions, nipples, scalp, vaccination sites, and the face.[11] Since patients with LS of face always complain the whitish plaque affecting the appearance, early diagnosis shows extremely important. However, disease of early stages is usually difficult to diagnose. The diagnosis of LS is usually clinical, combines with biopsy. Based on both of clinical and histologic features, the diagnosis of LS is made. Nowadays, dermoscopy and confocal microscopy are good choices for patients with face involvement without biopsy; they greatly help the diagnosis of LS if the patient refuses biopsy concerning the cosmetic impairment. The dermoscopy shows a whitish plaque with comedo-like openings on the surface of the lesion, while the confocal microscopy reveals hyperkeratosis, atrophy of the epidermis, dark and round structures containing bright amorphous material, and scattered inflammatory cells, as well as coarse collagen in bundles. All of these presences are correspond to the histopathologic features of epidermis atrophy, fibrosis of the upper dermis, and follicular plugging.[12] Hence, the diagnosis of extragenital LS can be made in noninvasive ways. However, it should also differentiate from morphea, vitiligo, atrophic lichen planus, and discoid lupus erythematosus.

To date, there are numerous therapies including oral and topical corticosteroids, topical calcineurin inhibitors (TCIs), estrogen, retinoids, various vitamins, phototherapy, surgical excision, and emollients are also recommended as a supportive treatment. However, it is not easy to make it complete resolution of clinical signs and return to normal skin texture and color, but therapy can help alleviate symptoms and prevent further progression. Oral corticosteroids combined with topical steroids or TCIs might be helpful to control the progress of the lesion. Hence, early diagnosis with dermoscopy or confocal microscopy and early treatment with oral corticosteroids combined with topical steroids or TCIs might cure it without hypopigmentation and atrophy. What's more, patients with classic LS of vulvar have about 0.3-4.9% risk developing squamous cell carcinoma (SCC).[3] While extragenital LS is not associated with the development of SCC,[13] few publications referring to the long-term follow-up of LS patients. Our review of the patients with LS involving face did not show any relationship with SCC and other cutaneous tumors. Compared with LS of vulva and mucosa, it may have less malignant potential. Though we still need to follow-up our patients, once suspicious, biopsied should also be made.

This study is limited by the number of cases, for reasons that some may under reporting, misdiagnosis as other diseases and asymptomatic presentations. However, dermatologists should also pay attention to this rarely reported manifestation of LS and consider it as a differential diagnosis. Early diagnosis with dermoscopy or confocal microscopy and early treatment might cure it without hypopigmentation and atrophy. It may have less malignant potential when compared with LS of vulva and mucosa, but we still need to offer long-term monitoring.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

What is new?

Our case demonstrated that though rare, face is also one presentation of LS, which we should not ignore. What's more, dermoscopy or confocal microscopy may help us early diagnosis without invasiveness, and it may have less malignant potential.