Indian Journal of Dermatopathology and Diagnostic Dermatology

ORIGINAL ARTICLE
Year
: 2017  |  Volume : 4  |  Issue : 1  |  Page : 8--13

Histopathological spectrum of lichen sclerosus Et atrophicus


Varsha Dalal1, Manveen Kaur1, Chandra Bhushan Rai1, Avninder Singh1, V Ramesh2,  
1 National Institute of Pathology, ICMR, New Delhi, India
2 Department of Dermatology, Vardhman Mahavir Medical College and Hospital, New Delhi, India

Correspondence Address:
Varsha Dalal
National Institute of Pathology, ICMR, Safdarjung Hospital Campus, New Delhi - 110 029
India

Abstract

Introduction: Lichen sclerosus (LS) et atrophicus is an inflammatory disorder of unknown etiology affecting skin and mucosa, especially the genital area. Clinically, its main features are whitish papules which converge to form plaques and atrophic patches. Histopathology of LS et atrophicus is characterized by the constellation of an atrophic epidermis with loss of rete ridges, some lymphocytes in the basal layer, a subepidermal band of sclerosis, and a lichenoid infiltrate of lymphocytes beneath that band is diagnostic of LS. Materials and Methods: Skin specimens from 25 patients with LS were collected from the hospital records for 5 years. The diagnosis of all cases was made on the basis of clinical morphology and histopathologic features. Sections were stained with hematoxylin and eosin, periodic acid-Schiff, and Elastic-Van Gieson. Criteria evaluated included hyperkeratosis, epidermal atrophy, follicular plugging, basal cell vacuolation, vascular ectasia, hyalinosis, inflammatory infiltrate, dermal edema, and deep dermal fibrosis. Results and Conclusion: Of a total of 25 patients, 18 patients had extragenital (EG) LS and 7 had genital manifestations. Mean age of patients with EG was 28 years, and genital was 38 years. To summarize, the main histopathological findings seen in LS are essentially the same as reported in literature, namely, hyperkeratosis, epidermal atrophy, follicular plugging, basal cell vacuolation, vascular ectasia, hyalinosis, inflammatory infiltrate, dermal edema, and deep dermal fibrosis. Moreover, some interesting differences between the EG and genital forms of LS were seen. However, since the figures are too small to comment on, studies comprising larger series of patients are required to bring out a statistical significance.



How to cite this article:
Dalal V, Kaur M, Rai CB, Singh A, Ramesh V. Histopathological spectrum of lichen sclerosus Et atrophicus.Indian J Dermatopathol Diagn Dermatol 2017;4:8-13


How to cite this URL:
Dalal V, Kaur M, Rai CB, Singh A, Ramesh V. Histopathological spectrum of lichen sclerosus Et atrophicus. Indian J Dermatopathol Diagn Dermatol [serial online] 2017 [cited 2020 Jan 21 ];4:8-13
Available from: http://www.ijdpdd.com/text.asp?2017/4/1/8/207567


Full Text

 Introduction



Lichen sclerosus (LS), et atrophicus, described originally by Hallopeau, in 1887 is an inflammatory disorder of unknown etiology affecting skin and mucosa, especially the genital area.[1],[2] Although reported in all age groups, a bimodal incidence is observed as it affects prepubertal girls and especially menopausal women.[3],[4] Clinically, its main features are whitish papules which converge to form plaques and atrophic patches. Histopathology of LS is characterized by alterations in the epidermis and dermis as well as inflammatory cell infiltration. The constellation of an atrophic epidermis with loss of rete ridges, some lymphocytes in the basal layer, a subepidermal band of sclerosis, and a lichenoid infiltrate of lymphocytes beneath that band is diagnostic of LS.[5],[6]

 Materials and Methods



The present study was retrospective wherein skin specimens from 25 patients with LS were collected from the hospital records over a period of 5 years. The diagnosis of all cases was made on the basis of clinical morphology and histopathologic features. Sections were stained with hematoxylin and eosin (H and E), periodic acid-Schiff (PAS), and Elastic-Van Gieson. Criteria evaluated included hyperkeratosis, epidermal atrophy, follicular plugging, basal cell vacuolation, vascular ectasia, hyalinosis, inflammatory infiltrate, dermal edema, and fibrosis.

 Observations and Results



Of a total of 25 patients, 18 patients had extragenital (EG) (Group I) LS and 7 had genital (Group II) manifestations [Table 1]. None of the patients had both genital as well as EG involvement. Mean age in Group I was 28 years, and Group II was 38 years. In Group I, the youngest and the oldest patient were of 1 year and 56 years, respectively; whereas in Group II, it was 4 years and 70 years, respectively. None of the patients had any past significant history or autoimmune disease. Serological studies for viral infections were negative. The most commonly affected sites were neck, back, and thigh in the EG region. One case each of the upper lip and breast were also present. Vulva and prepuce were the affected areas in genital region. The various histopathological features observed in all the cases have been tabulated in [Table 1] and [Table 2]. These findings were reported by the same set of observers to avoid any inter-observer variability.{Table 1}{Table 2}

The patients were followed up for 6 months to 2 years. The variable response was observed to treatment with high-potency steroids. None of the cases developed any hypertrophic or verrucous changes clinically thus, ruling out malignancy.

 Discussion



LS is a sclerosing inflammatory dermatosis, commonly affecting anogenital skin, with less common EG involvement varying from 15% to 20%.[2],[3],[4] However, the present study has more of EG cases. It is likely that a disease that is typically described in the genital area is not usually biopsied by the clinician who is fairly confident of this diagnosis.[5] In EG lesions, which have more clinical mimics, are frequently biopsied to confirm the diagnosis. The etiology of this disease is still unknown, and various factors such as hormonal, immunological alterations, genetic aberrations and Borrelia burgdorferi, hepatitis C virus and human papilloma virus (HPV) infection have been implicated.[7],[8] However, in all our cases, there was no significant family history or history of any autoimmune disease.

Our study confirms previous data suggesting that LS more commonly affects women than men. The male to female ratio in our study was 1:2. The mean age group affected was 31 years, 28 for EG, and 38 for genital lesions. This was lower than the age group reported by Sang et al.[9] Although this disease mainly affects middle-aged and elderly women, the youngest patient in the present study was a male infant. The most common locations for EG disease are the buttocks, thighs, breasts, submammary area, neck, back and chest, shoulders, axillae, and wrists.[8] The common sites in the present study were neck and back [Figure 1]. Genital lesions were commonly seen on the vulva and prepuce [Figure 2].{Figure 1}{Figure 2}

Typically, EG lesions are polygonal, bluish white papules which coalesce to become atrophic plaques. These lesions are prone to koebnerization and may express themselves in areas of physical trauma, continuous pressure, and scarring.[5],[8] In genital region, the main features are whitish papules which converge into plaques, often with erythema, ecchymosis, hyperkeratosis, fissures, excoriations, and telangiectasias. The mucosa is always spared so is the vagina and cervix in contrast to lichen planus. With progression, the skin atrophies and whitens, exhibiting a cigarette paper appearance. Symptoms include pruritus, burning, dyspareunia, dysuria, and painful defecation.[5],[10]

The literature mentions vacuolar interface reaction in conjunction with dermal sclerosis as the minimum diagnostic criterion for LS. This study highlights other histopathological findings that could support a diagnosis of LS and help avoid diagnostic errors. Salient histologic findings observed in our cases were as mentioned in [Table 1], [Table 2] and [Figure 1], [Figure 2]. In early lesions, these features may be quite subtle and may mimic those of psoriasis, lichen planus, or lichenoid dermatitis, thus making clinicopathologic correlation pivotal for the final diagnosis. In addition, these changes are often more prominent in adnexal structures than in interfollicular skin.[10] In the present study, hyperkeratosis was seen in 86% cases of genital and 61% cases of EG ones. Epidermal atrophy and thinning were more commonly seen in EG cases (93%), suggesting that EG lesions are more evolved.[11] Inflammation was observed in nearly all the genital lesions while dermal sclerosis was predominantly a feature of EG areas [Table 2] and [Figure 1], [Figure 2]. In routine H and E sections, the basement membrane may appear thickened because of the area of subepidermal sclerosis. However, PAS stain did not reveal any thickening in our cases [Figure 3]b. This is in contrast to the observation of Fung and LeBoit who noted thickening in 44% cases.[12]{Figure 1}{Figure 2}{Figure 3}

Vascular ectasia was the most commonly seen histomorphological features in all the cases. Our findings differ from that of Larre Borges et al., who found a greater predominance in genital cases.[7] These ectatic blood vessels along with the epidermal atrophy can also be caused due to the use of topical steroids. Although these features cannot be reliably attributed to be caused by the disease process or treatment, the use of steroids will also be accompanied by other changes such as reduction in both epidermal and dermis thickness, loss of sebaceous glands, and subcutaneous fat loss, none of which were seen in our cases.[13] LS usually shows a spectrum of vascular changes ranging from lymphocytic vasculitis to granulomatous phlebitis and leukocytoclastic vasculitis. The lymphocytic vasculitis may occur in the form of concentric lymphohistiocytic infiltrate, dense perivascular lymphohistiocytic cuffing with fibrin deposition, or intramurally in muscular vessels. Recent studies have identified autoantibodies to glycoprotein extracellular matrix protein 1, which may explain the histopathologic evidence of vasculitis and thickening of the blood vessels wall in LS.[2],[5] These findings were not seen in any of our case.

The dermis in LS is characterized by loss of elastic fibers leading to homogenization of upper dermis along with a band like lymphohistiocytic infiltrate and an increase of these fibers and fibrosis in lower dermis [Figure 3]a. The loss of elastic fibers in the upper dermis has been attributed to the degradation of elastic fibers (elastolytic change) by elastase-type proteases secreted from dermal fibroblasts or activated macrophages. On the other hand, an increase of elastin in the lower dermis may reflect a repair process.[14],[15] Morphea also shows deep dermal fibrosis but is not accompanied by the loss of elastic fibers in the papillary dermis as seen in LS [Table 3]. Although dermal hyalinosis/sclerosis is a diagnostic criterion for LS, it may be missing in early lesions.[6] Similarly, keratotic plugging may be more prevalent in the lesions of shorter duration.[7] However, no correlation existed between the duration of disease process and histological criteria as also reported by Marren et al.[16]{Table 3}

Inflammation primarily comprised lymphocytes and can be present in lichenoid or diffuse pattern beneath the zone of subepithelial edema. The inflammatory component can be a persistent or recurring phenomenon, thus has poor correlation with the duration or site of disease.[6] The CD3, CD8, and CD57 positive lymphocytic population is located just below the edematous dermis.[17] The infiltrate is initially dense, which along with the basal vacuolar degeneration raises the differential of lichen planus. The subsequent edema, loss of dermal elastin, paucity of cytoid bodies, and lack of wedge-shaped hypergranulosis are features specific to LS. Mycosis fungoides is another differential diagnosis sharing features such as epidermotropism and coarse collagen bundles in dermis [Table 3].[1],[2],[18]

Resolution of LS is usually difficult. However, in some cases, especially in prepubertal girls, it may remit spontaneously. Rarely, transformation into squamous cell carcinoma has been reported in 4%–6% of genital cases. Although the exact carcinogenic mechanism is still debatable, several HPV-independent hypotheses have been postulated.[1],[2],[4],[10] Given the long-term malignant potential, 6 months follow-ups and histopathological examination are recommended for all patients with genital LS, especially the clinically suspicious ones. In the present study, none of our biopsies showed any features of dysplasia or koilocytosis due to frequent association with autoimmune diseases such as alopecia, vitiligo, thyroid, and pernicious anemia; screening tests comprising blood tests for iron, B12, glucose, thyroid function, and autoantibody levels should be done for all patients.[4]

The treatment modality of choice is topical corticosteroids though EG lesions are less responsive than genital ones. The next most frequent, of controversial efficacy, are tacrolimus, pimecrolimus, calcipotriol, testosterone, retinoids, antimalarial agents, photodynamictherapy, and surgery. CO2 laser and oral stanozolol have also been reported as possible treatment modalities.[2],[4],[5]

 Conclusion



The main histopathological findings seen in LS are essentially the same as reported in literature, namely, hyperkeratosis, epidermal atrophy, follicular plugging, basal cell vacuolation, vascular ectasia, hyalinosis, inflammatory infiltrate, dermal edema, and sclerosis. Apart from this, we noticed some interesting differences between the EG and genital forms of LS. However, since the figures are too small to comment on, studies comprising larger series of patients are required to bring out a statistical significance.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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