Download Childhood lichen planus: A review of literature Research area: Oral

Childhood lichen planus: A review of literature
Research area: Oral medicine and pathology
a
Bassel tarakji, Alaa’ Melhemb
Corresponding author: a Head of Department of oral medicine and diagnostic science
Alfarabi College of dentistry, Kingdom of Saudi Arabia, Riyadh
Fax: 00966-2324580
Email: [email protected]
B
Lecturer at alfarabi dental school
Alfarabi College of dentistry, Kingdom of Saudi Arabia, Riyadh
Keywords: Lichen planus, children, premalignant lesion
Abstract: Oral lichen planus is a relatively common autoimmune mucocutaneousdisease affecting
between 0.5% and 2.2% of the population[1]. Lichen affects the skin, mucous membranes , nails and
hair and it was first described and named by the British physician Erasmus Wilson in 1869[2]. Skin
lesions in lichen planus usuallyresolve within 1-2 years where in oral lichen planus it persists for 20
years or more. The majority of patients are between 30 and 50 years of age and about 60% are
women .Oral lichen planus is also seen in children although rare[1,3,4].
The purpose of this article is to review the literature concerning oral lichen planus in childhood
which seems to be a rare entity in this group of patients and its reports are scarce [3, 5, 7-15].
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For that purpose a literature search using MEDLINE ,accessed via The National Library of
Medicine PubMed interface (http://www.ncbi.nlm.nih.gov/pubmed), searching for articles relating to
childhood oral lichen planus in English and using the following in search : (childhood oral lichen
planus , juvenile oral lichen plans ,oral lichen planus in pediatric patients , oral lichen planus in
children ). These references were obtained and from their bibliographies, secondary references were
also identified and acquired. The abstracted literature was reviewed.
Most of the studies were case reports or case series, no randomized controlled trials were found and
no statistical analysis is presented because the collected data were different and the results cannot be
compared.Studies that were reviewed are shown in Table 1.
Lichen planus is considered rare in children ( comprise 2-3% of reported cases[15], but it does not
appear to be uncommon in India and middle east countries with a study reporting an incidence of
7.5% childhood lichen planus among all registered lichen planus patients in the clinic[2,12,16,17].
Luis Montoya et al [18] in their study registered 24 cases of childhood lichen planus out of 235
lichen planus occurrences (10.2%) over a period of 22 years. It has been hypothized that the rarity of
associated autoimmune conditions, exposure to drugs and other triggers that have been known to
initiate lichen planus in adults may be responsible for the overall rarity of LP in children [2, 6].
In the majority of childhood lichen planus studies no significant gender predominance was
identified[18],however Sharma et al found the male: female ratio as 2:1 in their 50 childhood lichen
plans case series [17]. Kumar et al reported that more girls were affected and that was convenient
with Montoya et al[18], Handa et al [19]and Sanjaya et al[15].
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Etiopathogenesis:
Lichen planus is a unique disorder of unknown exact etiology and in most lichen planus cases the
precipitating factors are unknown and the disease is idiopathic ,however many factors have been
implicated in its uncertain etiology. Such factors include genetic predisposition(More recent studies
suggest that at least 50% of the cases reported , had familial histories of lichen planus), infective
agents , systemic diseases , graft vs. host disease , drug reactions , hypersensitivity to dental materials
and vitamin deficiencies .the association of HBV or HCV infection with lichen planus has been
given particular focus [20]. Lichen planus has also been associated with several auto-immune
diseases including lupus erythematosus,pemphigus, Sjogren’s syndrome and autoimmune liver
disease [5, 6, 16].
For childhood lichen planus no precipitating factors were observed in the study by Kanwar et al[2] as
well as by Handa et al[19] ,Sharma et al and Montoya el[18] but a number of childhood LP cases
were reported after hepatitis B vaccination and after combined measles-mumps-rubella
vaccination[2,16]. The family history of LP is more commonly positive in childhood than in
adulthood patients [6, 16].
The pathogenesis of lichen planus is not completely understood but there appear to be a mechanism
for activating the regional cellular immune response and another for the T- lymphocyte response that
eventuates in the destruction of the deep layered keratinocytes [1].
Clinical features:
Lesions occur on both cutaneous and oral surfaces comprise about 40% of all lichen planus cases,
lesions on cutaneous surfaces only comprise 35% and lesions on mucosal surfaces only are about
25% [1]
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The clinical presentation of lichen planus is diverse .The skin lesions present as purple, polygonal,
pruritic, flat topped papules. Wickham’s striae are evident on the surface of papule as a reticular
network of fine white lines. Oral lesions classically present as a bilaterally symmetrical white
network found on the buccal mucosa, tongue, lips and hard palate. Up to six clinical appearances of
oral lichen planus have been described [16].
Reticular oral lichen planus is the most common presentation, manifesting as a lacy network of
white striations. These lesions are often painless, although patients may complain of a slight
roughness or dryness of the affected sites. (Fig.1)
Papular oral lichen planus: manifests as small white raised areas approximately 1-2mm in
diameter. These again typically arise on the buccal mucosa and dorsum of tongue, although may also
present on other mucosal surfaces. Thisvariant may represent an early manifestation of the condition.
Plaque-like oral lichen planus: manifests as areas of homogenous whiteness. This typically arises
on the buccal mucosa or dorsum of tongue and may be more prevalent amongst those who are
smokers.
Atrophic oral lichen planus: There are areas of mucosal atrophy occur within the white patches.
The clinical picture is one of red and white areas, but not speckled as is seen in chronic hyperplastic
candidosis. Patients with this type of disease often complain of oral soreness.(Fig2.)
Ulcerative lichen planus frank ulcers exist often within the hyperkeratotic areas. On occasions there
may be no white striae apparent, making clinical diagnosis difficult. Patients complain of soreness,
particularly with spicy or acidic foods. (Fig3).
Bullous lichen planus is a rare presentation manifests as small vesicles or blister (bullae) within the
white patches (Fig. 4). This may cause diagnostic confusion with mucous membrane pemphigoid or
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pemphigus [1, 6, 15, and 25]. These variants may occur together in one patient or may transform
from one to another [6].
Oral mucosal involvement in adults itself is rare [15], and in children it seems to be even more rare
and very few of the children with cutaneous lesions were reported to have oral lichen planus [21]
Laeijendecker et al [6] in a study of 10 years duration (1994-2003) could identify three patients out
of 10000 ( with a ratio of .03%) having oral lichen planus , two of them were Asian .
In the study of Kumar et al [22] oral mucosal involvement with 20-nail dystrophy was detected in
only one patient among 25 children.
A study of 87 patients with childhood lichen planus for Handa et al [23] reported one case with oral
involvement alone, four cases with concomitant skin and mucosal involvement at the time of
presentation and seven patients with mucosal involvement later during the course of the disease.
Mucosal involvement was detected in three cases out of 13 children patients in a study of Nnoruka
[13] and in only one patient in Luis Montoya’s study [18].
Classic lichen planus was the commonest clinical variant [13, 14, 18], and Kobner phenomenon is
considered to be common in children with lichen planus [2]. Many authors contend that the clinical
features of oral lichen planus and the location are essentially the same as those in adults; however,
generally the prognosis of LP in childhood seems to be more favorable [6].
There have been sporadic reports in the literature that lichen planus may be responsible for the
subsequent development of squamous cell carcinoma and the percentage of this transformation was
estimated between 0.4% to 2% when lesions persist for more than 5 years [1, 15]. The scarcity of
literature in children does not allow for objective data regarding the malignant transformation rate in
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childhood lichen planus [6, 15].Nevertheless, the schedule of follow-up of oral lichen planus in
children should be at least one or two visits per year as long as oral lichen planus in children persists
and even more frequently in symptomatic childhood lichen planus, in line with oral lichen planus in
adults [6].
Histopathology:
In most cases of lichen planus it is not possible to make the correct diagnosis without a biopsy of the
perilesional tissue. The typical histological features of striae includes hyperkeratosis or
parakeratosis,saw–tooth profile of the rete ridges , liquifactive degeneration of the basal cell layer ,
compact band like T-cell infiltrate hugging the epithelio-mesenchymal junction and CD8
lymphocytes predominate in relation to the epithelium.(Fig.5) .
Treatment:
The aims of current oral lichen planus therapy are to eliminatemucosal erythema and ulceration,
alleviate symptoms and reduce the risk of oral cancer in oral lichen planus patients. Inthis context,
medical treatment is restricted currently to atrophic (erythematous), erosive (ulcerated), bullous
(blistering) or symptomatic oral lichen planus lesions [24].
In asymptomatic non ulcerative lichen planus an explanation for the patient about the nature of the
condition, including its etiology, clinical features, treatment, follow up and prognosis with an advice
on avoidance of risk factors and reassurance are the treatment guidelines [25].
Corticosteroids are the mainstay of OLP therapybecause of their activity in dampening cell
mediatedimmune activity and are administered topically, intralesionallyor systemically. The
combination of systemicand topical steroid therapy is often very effective.Localized oral lesions are
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treated with topical ointment, applied two to four times daily after meals. Thestrength and specific
preparations used need to be balanced carefully with the individual patient’s needs. Generalized oral
lesions are often treated effectively with a steroid mouth rinse twice daily after
meals.Intralesionaland perilesional injection of steroids is useful for persistent localized erosive OLP
lesions but should be used with due caution. Generalized atrophic or erosive oral lesions that do not
respond to topical therapy may be treated with a short course of systemiccorticosteroids [24].
Due to the lack of controlled clinical trials, no consensus exits regarding standardized therapy
regimens for LP in children [15]. In the general treatment of LP,oral antihistamines and topical
corticosteroids are the main therapeutic agents. [5, 6, 17] Topical tacrolimus was applied as a
treatment but several reports have shown the usefulness of it in the oral cavity [20]. A short course of
systemic steroids has been used in children to control widespread disease but most protocols using
topical andintralesional corticosteroids cause some adrenal suppression and clinicians need to be
aware of the precise amounts of these medications being used on a regular or irregular basis[24];
adverse effects must be considered carefully[5,17]. Dapsone, griseofulvin, PUVA (Psoralen and
ultraviolet A light photochemotherapy) and narrowband ultraviolet B photo therapy was also found to
be safe and effective [5, 14, 17, 21].
There is neither evidence base nor consensus as to the optimumreview interval. An annual review
interval of 1 year would seem reasonable formild forms of oral lichen planus, reducing to 6 monthly
for more severe forms and 3monthly if dysplasia is present on biopsy[25].
This study has shown that there is a lack ofpublished evidence regarding the management of oral
lichen planus at children.There is aconsensus that corticosteroids are the mainstay of oral lichen
planus therapybecause of their activity in dampening cell mediatedimmune activity and are
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administered topically, intralesionallyor systemically, although,there is dividedopinion regarding the
most appropriate treatmentfor oral lichen planus at children. The rarity of the disease in children, lack
of evidence and poor knowledge at general practitioners to diagnose this disease that might be
increased the malignant transformation rates at children versus adults. Randomized controlledclinical
trials could be used to address theseissues but are difficult to perform because of likelylow accrual
rates and the need for prolongedfollow-up times to assess clinical outcomes. The guideline of the
diagnosis and treatment of oral lichen planus at children should be clarified to all general
practitioners in the dental clinics to detect oral lichen planus early as much as possible to prevent of
the malignant transformation of that disease.
Conclusion:
It is recommended that although oral lichen planus is considered rare in childhood, the presence of
oral lesions should alert GP’s to include childhood lichen planus in their differential diagnosis of
other white lesions.
A double-blind randomized and controlled studies would be necessary for a safe and effective
treatment of childhood lichen planus.
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Legends of figures:
Fig1: Shows white lesion on the buccal mucosa which represent reticular form of oral lichen planus
Fig2: Shows white lesion with areas of red color within it on the lateral surface of the tongue and
diagnosed as atrophic oral lichen planus.
Fig3: Ulcer exists within a white area on the buccal mucosa and sulcus and diagnosed as ulcerative
type of oral lichen planus.
Fig4: Bulla within a white patch on the buccal mucosa diagnosed as bullous oral lichen planus
Fig5 : Histological features of lichen planus includes hyperkeratosis,saw–tooth profile of the rete
ridges , band like T-cell infiltrate hugging the epithelio-mesenchymal junction .
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