planus Asmita Parihar a , Sonal Sharma a, * , Sambit Nath Bhattacharya b , Usha Rani Singh a a Pathology, University College of Medical Sciences & GTB Hospital, Delhi, India b Dermatology, University College of Medical Sciences & GTB Hospital, Delhi, India Received 12 November 2013; revised 23 December 2013; accepted 23 December 2013 KEYWORDS Lichen planus; Cutaneous; Pathology Abstract Background: Lichen planus is an idiopathic subacute or chronic inammatory disease of the skin, mucous membranes and nails. We studied the clinicopathological prole of lichen planus in Indian population. Methods: A total of 145 cases of histologically diagnosed lichen planus samples were included. Clinical features like age, sex, type of lichen planus, location were recorded in the case record form. Histological features of lichen planus were studied. Results: Out of 145 cases, majority (61%) were of classical lichen planus. Majority of cases were in the age group of 2040 years and showed female preponderance. Most commonly, violaceous lesions occurred in lichen planus, pigmented in lichen planus pigmentosus, and both violaceous and pigmented in lichen planopilaris. Conclusions: In Indian population, common age of occurrence of lichen planus is lower as com- pared to western literature, and a large number of cases (28%) are in the paediatric age group (<18 years). 2014 Production and hosting by Elsevier B.V. on behalf of King Saud University. 1. Introduction Lichen planus (LP) is an idiopathic subacute or chronic inammatory disease of the skin, mucous membranes and nails. (Boyd and Neldner, 1991) Exact pathogenesis of lichen planus is still unclear. Several hypotheses have been made regarding its aetiology, including genetic, infective, psychogenic and autoimmune factors (Sugerman et al., 2000; Sontheimer, 2009). Recent studies provide evidence that autoreactive cytotoxic T lymphocytes are the effector cells which cause degeneration and destruction of keratinocytes (Sontheimer, 2009). * Corresponding author. Address: Department of Pathology, Uni- versity College of Medical Sciences & GTB Hospital, University of Delhi, Shahdara, Delhi 110095, India. Tel: +91 11 22592971x5612; fax: +91 11 22590495. E-mail address: sonald76@gmail.com (S. Sharma). Peer review under responsibility of King Saud University. Production and hosting by Elsevier Journal of the Saudi Society of Dermatology & Dermatologic Surgery (2014) xxx, xxxxxx King Saud University Journal of the Saudi Society of Dermatology & Dermatologic Surgery www.ksu.edu.sa www.jssdds.org www.sciencedirect.com 2210-836X 2014 Production and hosting by Elsevier B.V. on behalf of King Saud University. http://dx.doi.org/10.1016/j.jssdds.2013.12.003 Please cite this article in press as: Parihar, A. et al., Aclinicopathological study of cutaneous lichen planus. Journal of the Saudi Society of Dermatology & Dermatologic Surgery (2014), http://dx.doi.org/10.1016/j.jssdds.2013.12.003 Cutaneous lichen planus is characterized by polygonal at- topped, violaceous papules and plaques, which in some cases can be intensely itchy. The lesions may result in long-standing residual hyperpigmentation, especially in dark-skinned pa- tients. LP has characteristic histopathological features which make the diagnosis relatively easy. Cutaneous LP has worldwide distribution with its incidence varying from 0.22% to 1% depending upon geographic location (Boyd and Neldner, 1991). According to one study LP represents 0.38% of all dermatology outpatients in India. (Bhattacharya et al., 2000) However, there is a paucity of Indian literature describing the clinicopathological prole of lichen planus. Therefore, we conducted this study to document and analyse the clinicopathological prole of LP in Indian population. 2. Materials and methods The study was conducted in the Department of Pathology at our Institute. Retrospectively we analysed all the cases of lichen planus received in our department in the last 2 years. A total of 145 histologically diagnosed lichen planus samples were included for studying the clinicopathological aspects of lichen planus. Clinical features like age, sex, type of lichen planus, location were recorded in the case record form from the pathology archives. Haematoxylin and Eosin (H & E) stained slides and blocks were retrieved from the record for all cases. Sections stained with H & E were used to study the histological features of lichen planus. The diagnoses for all the lichen planus cases included in the study were conrmed on repeat histological examination. All morphological features were also noted for comparison with clinical subtype. 2.1. Statistical analysis Fishers Exact test was used to assess associations between var- ious variables. All analyses were performed using SPSS soft- ware. A p-value of less than 0.05 was considered statistically signicant. 3. Results Out of 145 cases of lichen planus studied, 88 (61%) were of li- chen planus classical type, 40 (27.5%) were of lichen planus pig- mentosus and 17 (11.5%) were of lichen planopilaris. 79/145 (54.5%) were females and 66/145 (45.5%) were males with sex ratio of 0.8:1 (male:female). Majority of the patients of lichen planus, lichen planus pigmentosus and lichen planopilar- is were in the age group of 2040 years. The age range of the pa- tients was from 5 to 60 years in males and 7 to 76 years in females. The mean age of males was a decade lower than that of females in LP and lichen planus pigmentosus (Figs. 1 and 2). The duration of the disease varied. Some patients sought help as early as a few days after its onset, while others, partic- ularly those with lichen planus pigmentosus had the disease for several years (Fig. 3). All the cases (145) were divided into different subgroups based on clinical variants as well as on the basis of anatomic distribution as involving head and neck, trunk , upper limb , lower limb or others (nail or mucosal involvement). Lesions of lichen planus (classical type) were mostly present on lower extremities while those of lichen planus pigmentosus and lichen planopilaris had head and neck as their predominant site of involvement (Fig. 4). A signicant association was found between the age of the patient and the occurrence of classical lichen planus on lower limb (p value = 0.001). It was observed that involvement of lower limb was more common in younger age group as 28/30 patients (93%) in the age group of less than 20 years had lower limb involvement. This association gradually decreased as the age increased. A signicant association was also seen between female gender and involvement of upper limb by lichen planus (p value = 0.031). Figure 3 Types and duration of lichen planus. Figure 1 Age and sex distribution of patients with different forms of lichen planus. Figure 2 Gender distribution according to type of disease. 2 A. Parihar et al. Please cite this article in press as: Parihar, A. et al., Aclinicopathological study of cutaneous lichen planus. Journal of the Saudi Society of Dermatology & Dermatologic Surgery (2014), http://dx.doi.org/10.1016/j.jssdds.2013.12.003 Cutaneous lesions were associated with mucosal lesion in 9% of cases and there were associated nail lesion in 5/145 (3.5%) of our patients. Clinical Characteristics and histopathological features (Fig. 5) Classical LP (n = 88): Lesions of 74/88 (84%) patients of classical LP were violaceous, 22/88 (25%) were pigmented, 10/88 (11%) were both violaceous and pigmented. Majority of the cases i.e. 75/88 (85%) showed regular inltration, while lesions of 6/88 (7%) were atrophic and 7/88 (8%) were hyper- trophic. Only 2/88 (2%) cases had eruptive lesions. Varying degrees of pruritus was seen as a manifestation in 80/88 (90%) patients of lichen planus. Figure 4 Anatomic distribution of different types of lichen planus. Figure 5 Clinical and histopathological features of lichen planus and its variants. (A) Clinical picture of lichen planus showing violaceous papules. (B) Photomicrograph of lichen planus showing band-like inltrate. (C) Clinical picture of lichen planus pigmentosus showing Pigmented lesions. (D) Photomicrograph of lichen planus pigmentosus showing epidermal thinning, basal layer vacuolation, pigment incontinence and mild perivascular inltrate. (E) Clinical picture of lichen planopilaris showing scarring alopecia and follicular plugging. (F) Photomicrograph of lichen planopilaris showing perifollicular inammation. A clinicopathological study of cutaneous lichen planus 3 Please cite this article in press as: Parihar, A. et al., Aclinicopathological study of cutaneous lichen planus. Journal of the Saudi Society of Dermatology & Dermatologic Surgery (2014), http://dx.doi.org/10.1016/j.jssdds.2013.12.003 Although there was some variability in histologic features, all of the sections examined were sufciently characteristic of lichen planus to verify the clinical impression. Orthokeratosis: All the cases 88/88 (100%) showed orthokeratosis. Acanthosis: Irregular acanthosis was seen in 83/88 (94%) cases. None of the cases had epidermal atrophy. Pointed rete ridges and dome shaped papillae were identi- ed in 67/88 (76%) cases. Hypergranulosis: 85/88 (96.5%) had wedge shaped hypergranulosis. Liquefaction degeneration is vacuolization of the basal layer of the epidermis resulting in loss of the usual morphology of the basal cells. This feature was present in at least a portion of each biopsy specimen. This may result in loss of cohesive- ness between the individual basal cells, and between the epider- mis and the dermis. Dermal inltrate: The inltrate in the upper dermis was band-like in 83/88 (94%) cases and in majority it was sharply demarcated at its lower border. The inltrate was composed almost entirely of lymphocytes intermingled with few histio- cytes. A few plasma cells could be seen in few cases. Civatte bodies or necrotic keratinocytes were present in 72/ 88 (82%) of cases in the lower epidermis and especially in the papillary dermis. They had a homogeneous, eosinophilic appearance and fair number of them also contained pyknotic or fragmented nuclei. They were PAS positive and diastase resistant. Pigment incontinence is a result of damage to the basal cells and was seen in 87/88 (99%) cases. Max Joseph spaces or small areas of artifactual separation between the epidermis and the dermis were apparent in 26/88 (29.5%) cases. Lichen planus pigmentosus (n = 40): majority of cases of lichen planus pigmentosus were in head and neck. Lesions of 9/40 (22.5%) patients were violaceous, 35/40 (87.5%) were pig- mented, 4/40 (10%) were violaceous and pigmented. Clinically, majority of the cases i.e. 38/40 (95%) showed regular inltration, while 2/40(5%) were atrophic and no case had hypertrophic lesions. Only 1/40 (2.5%) cases had eruptive lesions. Pruritus was seen in 3/40 (7.5%) patients of lichen planus pigmentosus. All the cases i.e. 40/40 (100%) showed epidermal thinning and pigment incontinence, and 34/40 (85%) cases showed ba- sal layer vacuolation. Lichen planopilaris (n = 17): majority of cases of lichen planopilaris were in head and neck. Lesions of 7/17 (41%) pa- tients were violaceous, 7/17 (41%) were pigmented, 2/17 (11%) were violaceous and pigmented. Clinically, 8/17 (47%) cases were atrophic, 9/17 (53%) showed regular inltration and no case had hypertrophic le- sions. No case had eruptive lesions. Histologically, all the cases 17/17 (100%) showed orthoker- atosis, wedge shaped hypergranulosis, perifollicular inltrate and basal layer vacuolation; 10/17 (59%) had civatte bodies; 4/17 (23.5%) showed follicular plugging and only 3/17 (17.6%) showed perifollicular brosis. Two more features were studied in lichen planopilaris, which were follicular plugging and alopecia. 4/17 (23.5%) patients had follicular plugging and 8/ 17(47%) patients had alopecia. 4. Discussion The present study describes the details of clinical and patho- logical characteristics of patients with lichen planus. In our ser- ies, we observed that classical lichen planus was the most common, constituting 61% of total cases followed by lichen planus pigmentosus (27.5%) and lichen planopilaris (11.5%). A similar dominance of classical lichen planus over other variants has been reported in the literature by various authors (Bhattacharya et al., 2000). In our series, maximum numbers of patients were seen in the age group of 2040 years. This correlates with other studies that describes data of Indian population (Bhattacharya et al., 2000; Singh and Kanwar, 1976) However in the western liter- ature (Andreason, 1968; Scully, 1985) an older age is reported. Difference in the age range between male and female was about a decade, which corresponds with western literature (Andreason, 1968; Scully, 1985). Furthermore, in the western literature LP is considered to be rare in children (Mellgren and Hersle, 1965) However, it is not uncommon in the Indian subcontinent (Kanwar and De, 2010). In our study we found that 41(28%) out of 145 pa- tients were less than 18 years of age. Out of these 41 patients only 13 patients had positive family history. This increased incidence of childhood LP in the Indian population as com- pared to western literature may be due to lack of data from other populations or due to some unidentiable environmental factors (Black, 1992). Although we found that children are more commonly affected with lichen planus in Indian population than wes- tern countries, but there was no statistically signicant correlation between any age group and occurrence of li- chen planus in Indian population, as we found that disease was uniformly present in all age group in Indian population. In our study we found that female gender is more com- monly affected with lichen planus than males. In the literature there has been no consistency regarding any sex preference of LP (White, 1919; Altman and Perry, 1961) but most of the studies have shown that females are more commonly affected than males (Little, 1919; White, 1919; Altman and Perry, 1961). In our series, we found that lower limbs (77.2%) were the most common site to be affected in classical lichen planus (Fig. 3). A similar observation has been reported in various studies and venous stasis has been offered as a likely explana- tion (Bhattacharya et al., 2000; Singh and Kanwar, 1976; Alt- man and Perry, 1961) No denite cause could be ascertained regarding the localization of lesions to lower limbs in our study. However, one interesting thing that we found is a statis- tically signicant association of involvement of lower limb in a younger age group (<20 years) (Table 1). Head and neck was the most common site of involvement in lichen planus pigmentosus and lichen planopilaris. A statis- tically signicant association was also seen between female gender and upper limb involvement. No such associations have been described in the literature till date to the best of our knowledge. Mucous membrane involvement with skin lesions in 9% of patients was low as compared to a reported involvement of mucous membrane in 1525% (Bhattacharya et al., 2000). 4 A. Parihar et al. Please cite this article in press as: Parihar, A. et al., Aclinicopathological study of cutaneous lichen planus. Journal of the Saudi Society of Dermatology & Dermatologic Surgery (2014), http://dx.doi.org/10.1016/j.jssdds.2013.12.003 The low incidence of mucous membrane involvement may be explained by the fact that in this institution, Department of Dermatology refers patients to the Department of E.N.T. for obtaining oral mucosal biopsies, and to the Department of Obstetrics and Gynaecology for obtaining genital mucosal biopsies. This may lead to unavoidable loss of data. Nail lesions were associated in 5/145 (3.5%) of our patients (Zaias, 1970). This is comparatively low, because reported involvement of nails range from 1 to 10%. In glabrous skin, the eruption of LP is characterized by small, at-topped, shiny, polygonal, violaceous papules that may coalesce into plaques. These ndings are very well pub- lished in the literature. Our study also supports these results as lesions of majority of our patients i.e. 74/88 (84%) were vio- laceous. Itching was associated with these lesions in 90% of cases. Most of the characteristic histopathologic features of LP were encountered with regularity in our study. Most frequently observed ndings were orthokeratosis, basal layer vacuolation, pigment incontinence, wedge shaped hypergranulosis, band like inltrate and irregular acanthosis. These changes were present in more than 90% of cases. Other ndings were those of civatte bodies, pointed rete ridges and dome shaped papillae which were seen in approximately 80% of cases. Max Joseph space was the least frequent nding, being present in only 30% cases. The results of our study correlates with that of Ellis et al (Ellis, 1967) except for the frequencies of irregular acanthosis, civatte bodies and Max Joseph space. The frequencies of these variables were higher in our study. 5. Lichen planus pigmentosus (LPP) LPP is a common pigmentary disorder seen in the Indian pop- ulation, having distinct clinical and histological characteristics as observed in the current study. Although distribution is variable, we observed that face and neck were the most frequent initial sites of involvement fol- lowed by the trunk. Upper and lower extremities were less fre- quently involved. Similar ndings have been reported in previous studies (Bhutani et al., 1974; Kanwar et al., 2003). Associated nail and mucosal involvement was infrequent. Majority of our patients (87.5%) had pigmented lesions. Pigmentation was mostly diffuse or reticular in pattern. This is in concordance with other studies (Kanwar et al., 2003) Pru- ritus was observed in 7.5% of our patients. This gure is lower than the reported frequency of 30% in previous studies (Kan- war et al., 2003). In our study, the histopathological changes most frequently observed in lichen planus pigmentosus were epidermal thin- ning and pigment incontinence in the dermis, seen in 100% of cases. Another nding was vacuolar degeneration of the ba- sal layer in the epidermis seen in 85% cases. 6. Lichen planopilaris Lichen planopilaris is an uncommon inammatory hair loss disease. The majority of the patients in our study were in the age group of 2040 years; an older age is reported in the western literature (Chieregato et al., 2003; Tan et al., 2004). Though female predominance has been reported in lichen planopilaris, the majority of our patients were males. The most common site involved was scalp, seen in 82% patients. Pruritus was associated in 17.5% of our cases; this is quite low compared to the reported gures of 70%. Equal number of cases [(7/17) 41%] of violaceous and pig- mented lesions were seen in this study. Atrophic lesions were seen in less than half of the cases. One report suggested that violaceous papules were observed in early lesions while late lesions were characterized by atrophic scarring areas (Chieregato et al., 2003). As reported, alopecia was observed in approximately half of our patients (Silver et al., 1953). In our study, the histopathological changes most frequently observed in lichen planopilaris were orthokeratosis, wedge shaped hypergranulosis, perifollicular inltrate and basal layer vacuolation, observed in 100% cases. Civatte bodies were seen in approximately 60% cases. Less frequently observed ndings were follicular plugging (23.5%) and perifollicular brosis (17.6%). Interfollicular epidermis was involved in 35% cases. 7. Conclusion Lichen planus is a disease of adults (2040 years) according to western data (Andreason, 1968; Scully, 1985) but in Indian population it is also common in paediatric age group (<18 years). The disease is relatively more common in females than males. Classical lichen planus has a strong association of involvement of the lower limbs in the younger age group. Involvement of upper limb is more common in female patients in lichen planus. Conict of interest None. References Altman, J., Perry, H.O., 1961. The variations and course of lichen planus. Arch. Dermatol. 84, 179191. Andreason, J., 1968. Oral Lichen Planus, A clinical evaluation of 115 cases. Oral Surg. 25 (31). Bhattacharya, M., Kaur, I., Kumar, B., 2000. Lichen planus: a clinical and epidemiological study. J. Dermatol. 27 (9), 576582, Sep. Bhutani, L.K., Bedi, T.R., Pandhi, R.K., Nayak, N.C., 1974. Lichen planus pigmentosus. Dermatologica 149, 4350. Black, M.M., 1992. Lichen planus and lichenoid eruption. 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Primary cicatricial alopecias: clinicopathology of 112 cases. J. Am. Acad. Dermatol. 50, 2532. White, C.J., 1919. Lichen Planus: a critical analysis of 64 cases. J. Cutan. Dis. 37, 671679. Zaias, N., 1970. The nail in lichen planus. Arch. Dermatol 101, 264. 6 A. Parihar et al. Please cite this article in press as: Parihar, A. et al., Aclinicopathological study of cutaneous lichen planus. Journal of the Saudi Society of Dermatology & Dermatologic Surgery (2014), http://dx.doi.org/10.1016/j.jssdds.2013.12.003