Lichen Planus Activity and Damage Index (LiPADI)

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1		Adam Reich	+ 1547 word(s)	1547	2021-12-23 04:33:51	\|
2	format correct	Nora Tang	Meta information modification	1547	2022-01-18 07:24:11	\| \|
3	format correct	Nora Tang	Meta information modification	1547	2022-01-21 09:06:45	\|

This entry is adapted from the peer-reviewed paper 10.3390/jcm11010023

Lichen planus (LP) is a chronic autoimmune disease that affects skin, oral and genital mucosa, and other sites.

assessment tool disease severity lichen planus

1. Introduction

Lichen planus (LP) is a chronic inflammatory mucocutaneous disease that affects about 0.5–2% of the general population ^[1]. The clinical presentation varies depending on the variant of LP and area involved, which may be skin, scalp, nails, and mucous membranes. Typical cutaneous LP is characterized by flat-topped, polygonal, violaceous papules and plaques ^[2]. Usually, the lesions are located on the trunk and flexor surfaces of the forearms, wrists, and ankles. While resolving, the lesions may also result in long-standing, residual hyperpigmentation. Some patients may demonstrate other clinical subtypes of LP, like hypertrophic, bullous, pigmented, linear, annular, atrophic, hypertrophic, inverse, eruptive, ulcerative, or actinic LP, LP pemphigoides, and overlap syndromes ^[2]. Clinical presentation of the rarer subtypes of LP may be dissimilar to classic LP; however, histopathological examination reveals consistent features, including band-like subepidermal lymphocytic inflammatory infiltrate and degeneration of the basal cell layer of the epidermis ^[2]^[3]. Lichen planopilaris is considered a follicular form of LP and classically presents as perifollicular erythema and scaling. This form of LP leads to irreversible destruction of hair follicle stem cells and to scarring alopecia ^[4]. When the nails are affected by LP, longitudinal ridging is the most common finding. Progression of the nail disease may result in dorsal pterygium and permanent nail destruction ^[5].

The most common affected mucosal sites for LP are oral and genital mucosae. Usually, mucosal lesions demonstrate a more chronic course than typical skin lesions. Oral LP is characterized by symmetric reticular whitish lines as well as by grouped papules and erosions on the buccal mucosa, tongue, soft palate, gingiva, and lips. Vulvovaginal LP most commonly affects postmenopausal women and may present as erosions surrounded by a white lacy border and whitish areas ^[6]. On the glans penis LP may present clinically as erythematous macules, papules, lacy network, and atrophic lesions ^[7].

The objective assessment of any disease severity is of great importance in dermatology and other medical specialties. Severity scales are essential to evaluate treatment outcomes in clinical trials and to compare the results of various studies. Furthermore, such scales are invaluable in clinical practice for overall judgment of disease severity, selecting a therapeutic option, and then guiding the course of treatment. Therefore, the measurements play important role in the therapeutic and research process. There are numerous severity scales available to evaluate various dermatologic diseases, the most widely used of which are e.g., SCORing Atopic Dermatitis (SCORAD) and Eczema Area and Severity Index (EASI) for assessment of atopic dermatitis ^[8], Psoriasis Area and Severity Index (PASI) and Body Surface Area (BSA) for assessment of psoriasis ^[9], and The Localized Scleroderma Assessment Tool (LoSCAT) for assessment of the severity of localized scleroderma (morphea) ^[10]. However, due to the variety of clinical manifestations and the coexistence of lesions in different locations, a comprehensive assessment of the severity of LP remains a challenge. To date, no appropriate tool for the overall assessment of LP has been proposed. Therefore, the objective of this study was to develop and validate a scoring system to assess the severity of LP in terms of activity and damage, taking into consideration various clinical features and different variants of the disease.

2.Lichen Planus Activity and Damage Index (LiPADI)

Increasing interest in the development of new therapies for many dermatological conditions in recent years implies the need for more accurate and reliable measurement of treatment outcomes. Thus, various assessment instruments are being prepared and validated for different skin diseases, e.g., psoriasis or atopic dermatitis. However, in some entities, the assessment might be challenging due to numerous clinical manifestations, different subjective sensations, and variable clinical courses with some persistent damages in selected patients. To our knowledge, the development of LiPADI is the first attempt to comprehensively assess the severity of LP in various locations, with particular emphasis on the skin.

Numerous scoring systems for the assessment of LP subtypes have been reported in the past; however, none of them have been recognized to date as a gold standard. For instance, more than 22 specific scoring systems were designed to grade the severity of oral LP ^[11], but these scales do not consider cutaneous lesions and have limited applicability for most LP patients, as they often present skin lesions. Of note, neither of these scales is universally accepted even for oral lesions, although the Thongprasom scoring system is most commonly used in clinical trials ^[12]. A similar situation may be observed in lichen planopilaris in which there have been some instruments developed for the assessment of disease severity ^[13]^[14]^[15], but they can be used only regarding lesions located within the skull area.

Interestingly, only two scoring systems have been proposed for cutaneous LP so far. In 2019, Bishnoi et al. ^[16] proposed Lichen Planus Activity, Area and Severity Index (LPAASI). The LPAASI has two components, one component which defines disease activity in terms of the progression of existing lesions and appearance of new lesions, and the second one which defines disease severity based on the morphology of the lesions, and extent of the disease considering the body surface area involved by LP ^[16]. Another scoring system for LP—Lichen Planus Severity Index (LPSI)—published in 2020, requires the calculation of the involved skin area using the rule of nines and lesion counting. To determine lichen planus severity, the counting of a total number of lesions and assessing the percentage of each of the morphological types (erythematous papules, violaceous papules, violaceous plaques, hyperpigmented hypertrophic papules and plaques, and post-inflammatory hyperpigmentation) is needed, followed by assignment of the area involvement factor. The final scoring requires multiplication with the multiplying factors for each respective lesion type and the total BSA factor ^[17]. It has to be mentioned that both scales are quite complicated, time-consuming, and difficult for implementation in routine daily practice, and only LPSI has been validated to some extend so far ^[16]^[17].

Here, we have proposed a new assessment tool for LP (LiPADI) which has been developed based on the scoring system of cutaneous lupus erythematosus (CLE)—CLASI—as, in our opinion, clinical manifestations of LP have a lot to do with the clinical manifestations of CLE. We have also provided some provisional data on the validity of LiPADI, but due to a limited number of analyzed patients and underrepresentation of some subtypes of LP, these results have to be considered with caution and need to be verified in future studies. We would like to underline the necessity to verify the utility of the scale particularly in LP limited to the mucous membranes, including both erosive and reticular/papular variants.

Nevertheless, we hope that it will be found easier and more comprehensive than previously developed instruments and may be implemented both in clinical trials as well as in routine daily practice. Similar to the assessment of CLE with CLASI, we divided the LP symptoms into two groups, namely, activity and damage, as we believe that some lesions in LP are just the consequences of the inflammatory process but do not necessarily reflect the current disease severity. However, they cannot be omitted, as it is important to have the comprehensive impact of the disease assessed in all patients. The essence of such division could be supported by the observations that damage scoring did not correlate with any assessments used in our study except for disease duration which can be easily explained by the fact that the longer the disease lasts, the more damage it causes. In contrast, the activity scoring significantly correlated with many aspects analyzed in our patients, such as global disease severity, quality of life, or pruritus severity. No significant difference of LiPADI activity scoring between various levels of lichen planus severity assessed by patients may be explained by the fact that such assessment done by the patient is very subjective. Sometimes, even limited lesions, e.g., lichen planopilaris, may be considered severe, because they may produce significant suffering due to pain or pruritus, and uncertainty caused by frequently unsuccessful therapies. We have also demonstrated that LiPADI is characterized by a good internal consistency and interrater reliability, which further supports its clinical applicability.

It has to be underlined that our study contains also several limitations. The major limitation is a rather small number of analyzed patients and we hope to continue the validation in the future, as assessing the validity of any scale is always an ongoing and long-lasting process. Furthermore, the studied population was not balanced regarding particular LP subtypes, and some variants were overrepresented, while many others were even absent. However, despite LP demonstrating significant clinical variability, many clinical variants are rare and it would be impossible to collect a representative group of patients with all clinical variants over a reasonable period of time. Finally, it would be valuable to further test the intra-rater reliability among the larger group of physicians to know the true variability of achieved results. Despite these limitations, we still consider that the new instrument is of value and worth being further tested in the next clinical trials.

In conclusion, the LiPADI has been proved to be an effective system for assessing disease severity in lichen planus showing satisfactory convergent validity, good test-retest reproducibility, high internal consistency, and some discriminating properties. We hope that, in the near future, LiPADI will be used also by other researchers.

References

Gupta, S.; Jawanda, M. Oral Lichen Planus: An Update on Etiology, Pathogenesis, Clinical Presentation, Diagnosis and Management. Indian J. Dermatol. 2015, 60, 222–229.
Weston, G.; Payette, M. Update on Lichen Planus and Its Clinical Variants. Int. J. Womens Dermatol. 2015, 1, 140–149.
Chhabra, S.; Saikia, U.; Dogra, S.; Minz, R.; Arora, S. Lichen Planus: A Clinical and Immuno-Histological Analysis. Indian J. Dermatol. 2014, 59, 257–261.
Errichetti, E.; Figini, M.; Croatto, M.; Stinco, G. Therapeutic Management of Classic Lichen Planopilaris: A Systematic Review. Clin. Cosmet. Investig. Dermatol. 2018, 11, 91–102.
Gupta, M.; Lipner, S. Review of Nail Lichen Planus. Dermatol. Clin. 2021, 39, 221–230.
Khurana, A.; Tandon, S.; Marfatia, Y.; Madnani, N. Genital Lichen Planus: An Underrecognized Entity. Indian J. Sex. Transm. Dis. AIDS 2019, 40, 105–112.
Amsellem, J.; Skayem, C.; Duong, T.; Bagot, M.; Fouéré, S.; Dauendorffer, J. Male Genital Lichen Planus: A Retrospective Study of 89 Cases. Ann. Dermatol. Venereol. 2021, in press. Available online: https://www.sciencedirect.com/science/article/abs/pii/S0151963821000569?via%3Dihub (accessed on 2 November 2021).
Bożek, A.; Reich, A. Assessment of Intra- and Inter-Rater Reliability of Three Methods for Measuring Atopic Dermatitis Severity: EASI, Objective SCORAD, and IGA. Dermatology 2017, 233, 16–22.
Bożek, A.; Reich, A. The Reliability of Three Psoriasis Assessment Tools: Psoriasis Area and Severity Index, Body Surface Area and Physician Global Assessment. Adv. Clin. Exp. Med. 2017, 26, 851–856.
Arkachaisri, T.; Vilaiyuk, S.; Torok, K.S.; Medsger, T.A., Jr. Development and Initial Validation of the Localized Scleroderma Skin Damage Index and Physician Global Assessment of Disease Damage: A Proof-of-Concept Study. Rheumatology 2010, 49, 373–381.
Wang, J.; van der Waal, I. Disease Scoring Systems for Oral Lichen Planus: A Critical Appraisal. Med. Oral Patol. Oral Cir. Bucal. 2015, 20, e199–e204.
Thongprasom, K.; Luangjarmekorn, L.; Sererat, T.; Taweesap, W. Relative Efficacy of Fluocinolone Acetonide Compared with Triamcinolone Acetonide in Treatment of Oral Lichen Planus. J. Oral Pathol. Med. 1992, 21, 456–458.
Chiang, C.; Sah, D.; Cho, B.; Ochoa, B.; Price, V. Hydroxychloroquine and Lichen Planopilaris: Efficacy and Introduction of Lichen Planopilaris Activity Index Scoring System. J. Am. Acad. Dermatol. 2010, 62, 387–392.
Holmes, S.; Ryan, T.; Young, D.; Harries, M. Frontal Fibrosing Alopecia Severity Index (FFASI): A Validated Scoring System for Assessing Frontal Fibrosing Alopecia. Br. J. Dermatol. 2016, 175, 203–207.
Saceda-Corralo, D.; Moreno-Arrones, Ó.; Fonda-Pascual, P.; Pindado-Ortega, C.; Buendía-Castaño, D.; Alegre-Sánchez, A.; Segurado-Miravalles, G.; Rodrigues-Barata, A.R.; Jaén-Olasolo, P.; Vaño-Galván, S. Development and Validation of the Frontal Fibrosing Alopecia Severity Score. J. Am. Acad. Dermatol. 2018, 78, 522–529.
Bishnoi, A.; Vinay, K.; Sendhil Kumaran, M.; Handa, S.; Parsad, D. Proposition of a Comprehensive Score to Assess the Disease Severity and Activity of Cutaneous Lichen Planus. Int. J. Dermatol. 2019, 58, e140–e142.
Kaur, H.; Nikam, B.; Jamale, V.; Kale, M. Lichen Planus Severity Index: A New, Valid Scoring System to Assess the Severity of Cutaneous Lichen Planus. Indian J. Dermatol. Venereol. Leprol. 2020, 86, 169.

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1. Introduction

2.Lichen Planus Activity and Damage Index (LiPADI)

References