- Email: [email protected]
A clinicopathological study of cutaneous lichen planus
Journal of the Saudi Society of Dermatology & Dermatologic Surgery (2014) xxx, xxx–xxx
King Saud University
Journal of the Saudi Society of Dermatology & Dermatologic Surgery www.ksu.edu.sa www.jssdds.org www.sciencedirect.com
ORIGINAL ARTICLE
A clinicopathological study of cutaneous lichen planus Asmita Parihar a, Sonal Sharma Usha Rani Singh a a b
a,*
b
, Sambit Nath Bhattacharya ,
Pathology, University College of Medical Sciences & GTB Hospital, Delhi, India 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 inflammatory disease of the skin, mucous membranes and nails. We studied the clinicopathological profile 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 20–40 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 compared to western literature, and a large number of cases (28%) are in the paediatric age group (<18 years). ª 2014 Production and hosting by Elsevier B.V. on behalf of King Saud University.
1. Introduction
* Corresponding author. Address: Department of Pathology, University College of Medical Sciences & GTB Hospital, University of Delhi, Shahdara, Delhi 110095, India. Tel: +91 11 22592971x5612; fax: +91 11 22590495. E-mail address: [email protected] (S. Sharma). Peer review under responsibility of King Saud University.
Production and hosting by Elsevier
Lichen planus (LP) is an idiopathic subacute or chronic inflammatory 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).
2210-836X ª 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., A clinicopathological 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
2
A. Parihar et al.
Cutaneous lichen planus is characterized by polygonal flattopped, 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 patients. 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 profile of lichen planus. Therefore, we conducted this study to document and analyse the clinicopathological profile 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 confirmed on repeat histological examination. All morphological features were also noted for comparison with clinical subtype.
Figure 1 Age and sex distribution of patients with different forms of lichen planus.
Figure 2
Gender distribution according to type of disease.
2.1. Statistical analysis Fisher’s Exact test was used to assess associations between various variables. All analyses were performed using SPSS software. A p-value of less than 0.05 was considered statistically significant. 3. Results Out of 145 cases of lichen planus studied, 88 (61%) were of lichen planus classical type, 40 (27.5%) were of lichen planus pigmentosus 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 planopilaris were in the age group of 20–40 years. The age range of the patients 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, particularly 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
Figure 3
Types and duration of lichen planus.
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 significant 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 significant association was also seen between female gender and involvement of upper limb by lichen planus (p value = 0.031).
Please cite this article in press as: Parihar, A. et al., A clinicopathological 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
A clinicopathological study of cutaneous lichen planus
3 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)
Figure 4 planus.
Anatomic distribution of different types of lichen
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 infiltration, while lesions of 6/88 (7%) were atrophic and 7/88 (8%) were hypertrophic. 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 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 infiltrate. (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 infiltrate. (E) Clinical picture of lichen planopilaris showing scarring alopecia and follicular plugging. (F) Photomicrograph of lichen planopilaris showing perifollicular inflammation.
Please cite this article in press as: Parihar, A. et al., A clinicopathological 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
4 Although there was some variability in histologic features, all of the sections examined were sufficiently 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 identified 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 cohesiveness between the individual basal cells, and between the epidermis and the dermis. Dermal infiltrate: The infiltrate in the upper dermis was band-like in 83/88 (94%) cases and in majority it was sharply demarcated at its lower border. The infiltrate was composed almost entirely of lymphocytes intermingled with few histiocytes. 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 pigmented, 4/40 (10%) were violaceous and pigmented. Clinically, majority of the cases i.e. 38/40 (95%) showed regular infiltration, 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 basal layer vacuolation. Lichen planopilaris (n = 17): majority of cases of lichen planopilaris were in head and neck. Lesions of 7/17 (41%) patients 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 infiltration and no case had hypertrophic lesions. No case had eruptive lesions. Histologically, all the cases 17/17 (100%) showed orthokeratosis, wedge shaped hypergranulosis, perifollicular infiltrate 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 fibrosis. 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.
A. Parihar et al. 4. Discussion The present study describes the details of clinical and pathological characteristics of patients with lichen planus. In our series, 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 20–40 years. This correlates with other studies that describes data of Indian population (Bhattacharya et al., 2000; Singh and Kanwar, 1976) However in the western literature (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 patients 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 compared to western literature may be due to lack of data from other populations or due to some unidentifiable environmental factors (Black, 1992). Although we found that children are more commonly affected with lichen planus in Indian population than western countries, but there was no statistically significant correlation between any age group and occurrence of lichen 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 commonly 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 explanation (Bhattacharya et al., 2000; Singh and Kanwar, 1976; Altman and Perry, 1961) No definite cause could be ascertained regarding the localization of lesions to lower limbs in our study. However, one interesting thing that we found is a statistically significant 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 statistically significant 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 15–25% (Bhattacharya et al., 2000).
Please cite this article in press as: Parihar, A. et al., A clinicopathological 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
A clinicopathological study of cutaneous lichen planus 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, flat-topped, shiny, polygonal, violaceous papules that may coalesce into plaques. These findings are very well published in the literature. Our study also supports these results as lesions of majority of our patients i.e. 74/88 (84%) were violaceous. 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 findings were orthokeratosis, basal layer vacuolation, pigment incontinence, wedge shaped hypergranulosis, band like infiltrate and irregular acanthosis. These changes were present in more than 90% of cases. Other findings 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 finding, 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 population, 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 followed by the trunk. Upper and lower extremities were less frequently involved. Similar findings 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) Pruritus was observed in 7.5% of our patients. This figure is lower than the reported frequency of 30% in previous studies (Kanwar et al., 2003). In our study, the histopathological changes most frequently observed in lichen planus pigmentosus were epidermal thinning and pigment incontinence in the dermis, seen in 100% of cases. Another finding was vacuolar degeneration of the basal layer in the epidermis seen in 85% cases. 6. Lichen planopilaris Lichen planopilaris is an uncommon inflammatory hair loss disease. The majority of the patients in our study were in the age group of 20–40 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.
5 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 figures of 70%. Equal number of cases [(7/17) 41%] of violaceous and pigmented 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 infiltrate and basal layer vacuolation, observed in 100% cases. Civatte bodies were seen in approximately 60% cases. Less frequently observed findings were follicular plugging (23.5%) and perifollicular fibrosis (17.6%). Interfollicular epidermis was involved in 35% cases. 7. Conclusion Lichen planus is a disease of adults (20–40 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. Conflict of interest None. References Altman, J., Perry, H.O., 1961. The variations and course of lichen planus. Arch. Dermatol. 84, 179–191. 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), 576–582, Sep. Bhutani, L.K., Bedi, T.R., Pandhi, R.K., Nayak, N.C., 1974. Lichen planus pigmentosus. Dermatologica 149, 43–50. Black, M.M., 1992. Lichen planus and lichenoid eruption. In: Champion, R.H., Ebling, F.J.G. (Eds.), Textbook of dermatology, 5th ed. Blackwell Scientific Publications, Oxford, pp. 1675–1695. Boyd, A.S., Neldner, K.H., 1991. Lichen planus. J. Am. Acad. Dermatol. 25 (4), 593–619. Chieregato, C., Zini, A., Barba, A., Magnanini, M., Rosina, P., 2003. Lichen planopilaris: report of 30 cases and review of the literature. Int. J. Dermatol. 42, 342–345. Ellis, F.A., 1967. Histopathology of lichen planus based on analysis of one hundred biopsies. J. Invest. Dermatol. 48, 143. Kanwar, A.J., De, D., 2010. Lichen planus in childhood: report of 100 cases. Clin. Exp. Dermatol. 35, 257–262. Kanwar, A.J., Dogra, S., Handa, S., Parsad, D., Radotra, B.D., 2003. A study of 124 Indian patients with lichen planus pigmentosus. Clin. Exp. Dermatol. 28 (5), 481–485, Sep. Little, E.G., 1919. Lichen Planus. J. Cutan. Dis. 37, 639. Mellgren, L., Hersle, K., 1965. Lichen planus––a clinical study with statistical methods. Ind. J. Dermatol. 11, 1. Scully, C., EL-Kom, M., 1985. Planus- review and update on pathogenesis. Oral. Pathol. 14, 431–458.
Please cite this article in press as: Parihar, A. et al., A clinicopathological 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
6 Silver, H., Chargin, L., Sachs, P.M., 1953. Follicular lichen planus (lichen planopilaris). AMA Arch. Derm. Syphilol 67 (4), 346–354. Apr. Singh, O.P., Kanwar, A.J., 1976. Lichen planus in India: an appraisal of 441 cases. Int. J. Dermatol. 15, 752–756, Dec. Sontheimer, R.D., 2009. Lichenoid tissue reaction/Interface dermatitis: clinical and histological perspectives. J. Invest. Dermatol. 129 (5), 1088–1099.
A. Parihar et al. Sugerman, P.B., Satterwhite, K., Bigby, M., 2000. Autocytotoxic Tcell clones in lichen planus. Br. J. Dermatol. 142 (3), 449–456. Tan, E., Martinka, M., Ball, N., Shapiro, J., 2004. Primary cicatricial alopecias: clinicopathology of 112 cases. J. Am. Acad. Dermatol. 50, 25–32. White, C.J., 1919. Lichen Planus: a critical analysis of 64 cases. J. Cutan. Dis. 37, 671–679. Zaias, N., 1970. The nail in lichen planus. Arch. Dermatol 101, 264.
Please cite this article in press as: Parihar, A. et al., A clinicopathological 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
King Saud University
Journal of the Saudi Society of Dermatology & Dermatologic Surgery www.ksu.edu.sa www.jssdds.org www.sciencedirect.com
ORIGINAL ARTICLE
A clinicopathological study of cutaneous lichen planus Asmita Parihar a, Sonal Sharma Usha Rani Singh a a b
a,*
b
, Sambit Nath Bhattacharya ,
Pathology, University College of Medical Sciences & GTB Hospital, Delhi, India 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 inflammatory disease of the skin, mucous membranes and nails. We studied the clinicopathological profile 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 20–40 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 compared to western literature, and a large number of cases (28%) are in the paediatric age group (<18 years). ª 2014 Production and hosting by Elsevier B.V. on behalf of King Saud University.
1. Introduction
* Corresponding author. Address: Department of Pathology, University College of Medical Sciences & GTB Hospital, University of Delhi, Shahdara, Delhi 110095, India. Tel: +91 11 22592971x5612; fax: +91 11 22590495. E-mail address: [email protected] (S. Sharma). Peer review under responsibility of King Saud University.
Production and hosting by Elsevier
Lichen planus (LP) is an idiopathic subacute or chronic inflammatory 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).
2210-836X ª 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., A clinicopathological 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
2
A. Parihar et al.
Cutaneous lichen planus is characterized by polygonal flattopped, 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 patients. 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 profile of lichen planus. Therefore, we conducted this study to document and analyse the clinicopathological profile 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 confirmed on repeat histological examination. All morphological features were also noted for comparison with clinical subtype.
Figure 1 Age and sex distribution of patients with different forms of lichen planus.
Figure 2
Gender distribution according to type of disease.
2.1. Statistical analysis Fisher’s Exact test was used to assess associations between various variables. All analyses were performed using SPSS software. A p-value of less than 0.05 was considered statistically significant. 3. Results Out of 145 cases of lichen planus studied, 88 (61%) were of lichen planus classical type, 40 (27.5%) were of lichen planus pigmentosus 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 planopilaris were in the age group of 20–40 years. The age range of the patients 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, particularly 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
Figure 3
Types and duration of lichen planus.
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 significant 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 significant association was also seen between female gender and involvement of upper limb by lichen planus (p value = 0.031).
Please cite this article in press as: Parihar, A. et al., A clinicopathological 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
A clinicopathological study of cutaneous lichen planus
3 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)
Figure 4 planus.
Anatomic distribution of different types of lichen
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 infiltration, while lesions of 6/88 (7%) were atrophic and 7/88 (8%) were hypertrophic. 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 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 infiltrate. (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 infiltrate. (E) Clinical picture of lichen planopilaris showing scarring alopecia and follicular plugging. (F) Photomicrograph of lichen planopilaris showing perifollicular inflammation.
Please cite this article in press as: Parihar, A. et al., A clinicopathological 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
4 Although there was some variability in histologic features, all of the sections examined were sufficiently 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 identified 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 cohesiveness between the individual basal cells, and between the epidermis and the dermis. Dermal infiltrate: The infiltrate in the upper dermis was band-like in 83/88 (94%) cases and in majority it was sharply demarcated at its lower border. The infiltrate was composed almost entirely of lymphocytes intermingled with few histiocytes. 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 pigmented, 4/40 (10%) were violaceous and pigmented. Clinically, majority of the cases i.e. 38/40 (95%) showed regular infiltration, 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 basal layer vacuolation. Lichen planopilaris (n = 17): majority of cases of lichen planopilaris were in head and neck. Lesions of 7/17 (41%) patients 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 infiltration and no case had hypertrophic lesions. No case had eruptive lesions. Histologically, all the cases 17/17 (100%) showed orthokeratosis, wedge shaped hypergranulosis, perifollicular infiltrate 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 fibrosis. 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.
A. Parihar et al. 4. Discussion The present study describes the details of clinical and pathological characteristics of patients with lichen planus. In our series, 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 20–40 years. This correlates with other studies that describes data of Indian population (Bhattacharya et al., 2000; Singh and Kanwar, 1976) However in the western literature (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 patients 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 compared to western literature may be due to lack of data from other populations or due to some unidentifiable environmental factors (Black, 1992). Although we found that children are more commonly affected with lichen planus in Indian population than western countries, but there was no statistically significant correlation between any age group and occurrence of lichen 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 commonly 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 explanation (Bhattacharya et al., 2000; Singh and Kanwar, 1976; Altman and Perry, 1961) No definite cause could be ascertained regarding the localization of lesions to lower limbs in our study. However, one interesting thing that we found is a statistically significant 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 statistically significant 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 15–25% (Bhattacharya et al., 2000).
Please cite this article in press as: Parihar, A. et al., A clinicopathological 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
A clinicopathological study of cutaneous lichen planus 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, flat-topped, shiny, polygonal, violaceous papules that may coalesce into plaques. These findings are very well published in the literature. Our study also supports these results as lesions of majority of our patients i.e. 74/88 (84%) were violaceous. 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 findings were orthokeratosis, basal layer vacuolation, pigment incontinence, wedge shaped hypergranulosis, band like infiltrate and irregular acanthosis. These changes were present in more than 90% of cases. Other findings 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 finding, 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 population, 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 followed by the trunk. Upper and lower extremities were less frequently involved. Similar findings 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) Pruritus was observed in 7.5% of our patients. This figure is lower than the reported frequency of 30% in previous studies (Kanwar et al., 2003). In our study, the histopathological changes most frequently observed in lichen planus pigmentosus were epidermal thinning and pigment incontinence in the dermis, seen in 100% of cases. Another finding was vacuolar degeneration of the basal layer in the epidermis seen in 85% cases. 6. Lichen planopilaris Lichen planopilaris is an uncommon inflammatory hair loss disease. The majority of the patients in our study were in the age group of 20–40 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.
5 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 figures of 70%. Equal number of cases [(7/17) 41%] of violaceous and pigmented 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 infiltrate and basal layer vacuolation, observed in 100% cases. Civatte bodies were seen in approximately 60% cases. Less frequently observed findings were follicular plugging (23.5%) and perifollicular fibrosis (17.6%). Interfollicular epidermis was involved in 35% cases. 7. Conclusion Lichen planus is a disease of adults (20–40 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. Conflict of interest None. References Altman, J., Perry, H.O., 1961. The variations and course of lichen planus. Arch. Dermatol. 84, 179–191. 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), 576–582, Sep. Bhutani, L.K., Bedi, T.R., Pandhi, R.K., Nayak, N.C., 1974. Lichen planus pigmentosus. Dermatologica 149, 43–50. Black, M.M., 1992. Lichen planus and lichenoid eruption. In: Champion, R.H., Ebling, F.J.G. (Eds.), Textbook of dermatology, 5th ed. Blackwell Scientific Publications, Oxford, pp. 1675–1695. Boyd, A.S., Neldner, K.H., 1991. Lichen planus. J. Am. Acad. Dermatol. 25 (4), 593–619. Chieregato, C., Zini, A., Barba, A., Magnanini, M., Rosina, P., 2003. Lichen planopilaris: report of 30 cases and review of the literature. Int. J. Dermatol. 42, 342–345. Ellis, F.A., 1967. Histopathology of lichen planus based on analysis of one hundred biopsies. J. Invest. Dermatol. 48, 143. Kanwar, A.J., De, D., 2010. Lichen planus in childhood: report of 100 cases. Clin. Exp. Dermatol. 35, 257–262. Kanwar, A.J., Dogra, S., Handa, S., Parsad, D., Radotra, B.D., 2003. A study of 124 Indian patients with lichen planus pigmentosus. Clin. Exp. Dermatol. 28 (5), 481–485, Sep. Little, E.G., 1919. Lichen Planus. J. Cutan. Dis. 37, 639. Mellgren, L., Hersle, K., 1965. Lichen planus––a clinical study with statistical methods. Ind. J. Dermatol. 11, 1. Scully, C., EL-Kom, M., 1985. Planus- review and update on pathogenesis. Oral. Pathol. 14, 431–458.
Please cite this article in press as: Parihar, A. et al., A clinicopathological 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
6 Silver, H., Chargin, L., Sachs, P.M., 1953. Follicular lichen planus (lichen planopilaris). AMA Arch. Derm. Syphilol 67 (4), 346–354. Apr. Singh, O.P., Kanwar, A.J., 1976. Lichen planus in India: an appraisal of 441 cases. Int. J. Dermatol. 15, 752–756, Dec. Sontheimer, R.D., 2009. Lichenoid tissue reaction/Interface dermatitis: clinical and histological perspectives. J. Invest. Dermatol. 129 (5), 1088–1099.
A. Parihar et al. Sugerman, P.B., Satterwhite, K., Bigby, M., 2000. Autocytotoxic Tcell clones in lichen planus. Br. J. Dermatol. 142 (3), 449–456. Tan, E., Martinka, M., Ball, N., Shapiro, J., 2004. Primary cicatricial alopecias: clinicopathology of 112 cases. J. Am. Acad. Dermatol. 50, 25–32. White, C.J., 1919. Lichen Planus: a critical analysis of 64 cases. J. Cutan. Dis. 37, 671–679. Zaias, N., 1970. The nail in lichen planus. Arch. Dermatol 101, 264.
Please cite this article in press as: Parihar, A. et al., A clinicopathological 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