Extragenital Lichen Sclerosus: A Retrospective Study of 17 Patients.

Introduction: Lichen sclerosus is a chronic inflammatory and atrophic dermatosis affecting preferentially the anogenital region. However, the cutaneous involvement remains less known and studied.
Methods: We collected 17 patients to study the clinical and therapeutic features of cutaneous lichen sclerosus.
Results: We noticed that the frequency of extragenital involvement in our series is high (about 40%). There is a female predominance (76%), with two infantile cases presenting a severe involvement. On the other hand, the absence of sclerosis, in early forms, does not eliminate the diagnosis. Moreover, breast involvement was frequent (41%) and atypical locations, such as the face, were reported. There was an equal frequency between the diffuse and the localized forms. A genital involvement must imperatively be sought. Conclusions: Our series mention the frequency of isolated cutaneous lichen sclerosus. Clinical presentation can be misleading in the early forms because of lack of sclerosis, variability of localizations, variability of severity, and the absence of anogenital lichen sclerosus. Copyright:

Introduction

Lichen sclerosus (LS) is a chronic inflammatory dermatosis, with a tropism for the anogenital region. However, the cutaneous involvement is rare and represents a source of esthetic prejudice. Therefore, its treatment is not codified. For this reason, our series aims at studying the clinical, histological, and therapeutic particularities of cutaneous LS (CLS).

Methods

Our retrospective study gathers all the LSC cases diagnosed during a ten-year period (2011–2020). For this purpose, we collected anamnestic, clinical, histological, and therapeutic data.

Results

During the ten years of the study, of the 42 cases of LS diagnosed, 17 patients (13 women and 4 men, including 2 children) had CLS. In addition, the median age at diagnosis was 44 years with extremes ranging from 4 to 83 years. The median duration of evolution was two years. Only one woman had a personal history of vitiligo without any family history. Moreover, five women were menopausal. Then, lesions were ivory-white, shiny macules with an erythematous border in five cases, while sclerosis and atrophy were initially missing in seven cases (41%). In fact, the size of the lesions was variable between small plaques (lesions less than 1 cm) (in eight patients: 47%), larger patches (in seven patients: 41%), or as a combination of both aspects in two patients (12%). However, linear form was found in only one patient. The disease was localized in nine cases with involvement of the breasts (five cases) [Figure 1], including one at the areola and one in the sub-mammary area, the back (two cases), the neck (one case), and the forearm (one case). Moreover, the disease was diffuse in the other eight patients (47%) [Figure 2(a)]. Only one patient had facial involvement (forehead) [Figure 2(b)]. Pruritus was observed in five cases. A genital LS was found in four women (23%). Nevertheless, no functional repercussions were observed despite the presence of discomfort in the atrophic forms. A skin biopsy confirmed the diagnosis of LS in all cases. Thus, the predominant histological aspects consist of epidermal atrophy, orthokeratotic hyperkeratosis, hydropic changes of the basal layer, dermal edema, band inflammatory infiltrate, loss of elastic fibers, and sclerosis with a thickening of the collagen fibers localized in the papillary dermis [Figure 3]. For treatment, the nine patients with localized involvement received strong-class dermocorticoids (DC) combined with calcipotriol in two cases, with partial improvement (percentage of healing area less than 50%). For patients with diffuse CLS, we opted for acitretin in three cases [Figure 2(c)] (with general corticosteroid in one case). The evolution was good. On the other hand, chloroquine was prescribed in one case but without any improvement. In fact, all the results are summarized in Table 1.

Figure 1

Breast involvement: small lesions and a larger patch with telangiectasia

Figure 2

(a) Diffuse CLS in a child, (b) Involvement of the forehead, (c) Significant improvement after acitretin

Figure 3

Histopathological image: epidermal atrophy with orthokeratotic hyperkeratosis and hydropic changes of the basal layer. Replacement of papillary dermis by hyalinized and edematous hypocellular band with chronic inflammatory infiltrate. (H&E, ×100)

Table 1

Summary of the epidemiological, clinical and therapeutic characteristics of the 17 patients included in the CLS series

Epidemiological characteristics
Sex	Female	13: 76,5%
	Male	4: 23,5%
Age	Median	44 years
	Extreme	4–84 years
	Children	2 (4 et 10 years)
Duration of development	Median	2 years
	Extreme	1 month and 15 years
Personal history of autoimmunity	Vitiligo	1 case
Clinical characteristics
Clinical presentation	Hypochromia	17 cases
	Shiny aspect	6 cases
	Erythematous border	5 cases
	Atrophy	10 cases
	Telangiectasia	1 case
Clinical form	Small plaques	8 cases
	Large patches	7 cases
	Both association	2 cases
	Linear	1 case
Severity	Localized	9 cases: 53%
	Diffuse	8 cases: 47%
Localization	Back (+ shoulders)	8 cases: 47%
	Breasts	7 cases: 41% (one at the areola and one in the submammary area)
	Upper members	5 cases: 29%
	Lower limbs	5 cases: 29%
	Low-necked	3 cases: 17%
	Neck	1 case: 5%
	Abdomen	1 case: 5%
	Face	1 case (forehead): 5%
Symptoms	Itching	5 cases: 29%
	Functional gene	0 cases
Associations	Genital lichen sclerosus	4 women
	Oral lichen	0 cases
Therapeutic characteristics
Treatment	Number of cases	Results
Dermocorticoids	7	Partial improvement
Combined therapy: Dermocorticoids + calcipotriol	2	Partial improvement
Acitretin	2	Significant improvement
Combined therapy: acitretin + general corticosteroid	1	Significant improvement
Chloroquine	1	No improvement

Discussion

Lichen sclerosus (LS) is a chronic inflammatory dermatosis with a particular tropism for the genital area. However, extragenital involvement is rare (in 6–20%), and isolated (in 2.5–6%).[1-3] In our series, we found a high rate of CLS (40%), often associated with genital LS in 23%. Actually, LS occurs mainly in postmenopausal women which supports the hormonal hypothesis. Besides, it can affect young subjects, men, and even children at any age, as shown in our series. Moreover, the genetic background (HLA II DQ7) and autoimmunity (21.5–34% of cases)[4] were found in only one case in our series, in a woman with vitiligo. However, no familial case was found. As for the other described etiopathogenic factors, which are infectious, they were not investigated in our patients due to the absence of warning signs.

In fact, clinically, the CLS appears as white patches and it may be atrophic. Common extragenital sites of involvement are trunk, breasts, submammary folds, neck, shoulders, buttocks, and thighs.[2] All these locations were found in our series; besides, breast involvement was frequent (41%). The areola involvement, which was observed in one of our patients, was rarely reported[4]. Atypical locations would be the palms, soles, scalp, and the face which were found in our series. Trauma and friction may cause Koebner's phenomenon triggering LS and are considered as etiological factors in the LS located at radiation sites, surgical scars or vaccination sites.[25] The severity of involvement, which was highly variable with an equal frequency between the diffuse and localized forms in our series, in contrast to the rarity of the diffuse forms in the literature. Both children have a diffuse form. Genital localization must imperatively be sought. An extragenital LS is less symptomatic than a genital one,[2] like pruritus is inconsistent in our series. The similarity of clinical appearance and location with lichen planus were noticed suggesting that both entities belong to the same pathological spectrum.[6] Besides, rarer forms such as blaschko linear, bullous, and hemorrhagic, were reported.[789] On the other hand, the functional impact of the atrophy, which can manifest itself as limitation of mobility in severe forms, was not found in our series.

In fact, since sclerosis in its early forms may be missing, its diagnosis may be difficult. Therefore, its diagnosis relies on skin histology, which revealed atrophy of the epithelium, with horizontalization of the basal area, follicular hyperkeratosis, and specifically the presence of a sub-epithelial band composed of fibrous or edematous collagen in the superficial dermis with lacking elastic fibers.[3] These cases with a clinical appearance of vitiligo and histological features of LS were considered as “vitiligoid LS” by some authors.[10] Then, the histological differences with the superficial morphea are the loss of elastic fibers in the superficial dermis and the vacuolization of the basal layer in the CLS.

The treatment of CLS, which is not codified, may include local treatment (potent topical DC or intralesional corticosteroid, calcipotriol, tacrolimus), more or less associated with a general treatment (synthetic antimalarials, retinoids, general corticosteroid therapy, methotrexate or cyclosporine) depending on the evolution of the disease under a local treatment. Indeed, a recent series has examined the efficacy of methotrexate in forms resistant to topical treatment.[11] In our series, the improvement under DC of localized forms is partial, which implies that acitretin seems to be efficient in diffuse forms. On the other hand, chloroquine, which was prescribed to only one of our patients, was not efficient. UVB phototherapy can also be useful in refractory forms.[2] Unlike genital LS, extragenital LS is not associated with a risk of carcinomatous transformation.[13]

To conclude, we can say that our series is characterized by the frequency of isolated cutaneous LS involvement, the rarity of the autoimmune field, the lack of sclerosis in the early forms, the frequency of the breast localization, the involvement of the areola and the face, which were rarely reported, as well as the frequency of the diffuse form. Therefore, the treatment is based on the DC in localized forms. On the other hand, acitretin was effective in severe forms.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.