CASE REPORT
Orthodontic Treatments of Papillon-Lefevre Syndrome: Two
Case Reports
Laila Fawzi Baidas
BDS, FDS, MSc
Papillon-Lefevre syndrome (PLS) is a rare autosomal recessive disorder characterized by palmoplantar hyperkeratosis and early
onset of severe destructive periodontitis causing premature loss of both deciduous and permanent dentitions at a young age.
In this article two cases of patients with Papillon-Lefevre syndrome in late mixed dentition are presented. The objective of these
case reports was to illustrate that under a controlled regime of periodontal treatment, orthodontic treatment is possible in patients
with Papillon-Lefevre syndrome. In both cases, the deciduous dentition was lost prematurely shortly after eruption. The permanent
teeth erupt without any guidance, and this can lead to loss of space, crowding, and collapse of the dental arch. The aim of the
treatment was to expand the arch, create space to allow normal eruption of the permanent teeth, and stabilize the occlusion to
help the patient achieve a normal facial appearance rather than the collapsed appearance caused by early extractions.
KEYWORDS: Papillon-Lefevre Syndrome, Orthodontics, Mixed dentition, Case reports
HOW TO CITE: Baidas LF. Orthodontic treatments of papillon-lefevre syndrome: Two case reports. J Pak Dent Assoc
2021;30(2):132-138.
DOI: https://doi.org/10.25301/JPDA.302.132
Received: 25 November 2020, Accepted: 01 February 2021
INTRODUCTION
P
apillon-Lefevre Syndrome (PLS) is a rare autosomal
recessive disorder.1 It was first discovered in 1924
by French physicians Papillon and Lefevre. It is
characterized by a palmoplantar hyperkeratosis, and early
onset of periodontitis in the deciduous and permanent
dentitions.2 Reviews of the literature have focused on the
syndrome's genetic basis, 3,4 as well as its periodontal
management.5-6 It has been reported that consanguineous
offspring have greater frequency of occurrence of the
syndrome due to genetic predisposition. The prevalence of
PLS is 1-4 cases per million people with no racial or sex
predominance, and the carrier frequency appears to be 2-4
per thousand population.7 The identified genetic defect in
PLS is located on chromosome 11q14.14.3 as a mutation
of the cathepsin C gene.8 Previous studies showed that a
90% reduction of the cathepsin C gene causes a deficiency
of cathepsin C enzymatic activity, resulting in reduced
immunity and host response against bacteria.9,10 The cathepsin
C gene is found in epithelial regions, keratinized oral gingiva,
and various immune cells and their precursors.11 Even with
these advances in recognizing the genetic predisposition of
the syndrome, the pathogenesis leading to the periodontal
involvement is still unclear.12
Dermatological signs develop before the age of 4 to 6
months and remain throughout the patient's life. Common
Consultant and Associate Professor, Department of Pediatric Dentistry and Orthodontics,
College of Dentistry, King Saud University, Saudi Arabia.
Corresponding author: “Dr. Laila Fawzi Baidas” < lbaidas@ksu.edu.sa >
dermatological changes include well-demarcated
erythematous hyperkeratotic lesions on the soles, the palms
and the dorsum of the hand.7 Periodontitis could affect the
primary and permanent dentitions resulting in premature
tooth loss of both dentitions. Classically, eruption of the
primary dentition into the oral cavity is accompanied by
severe gingival inflammation and subsequent rapid
destruction of the periodontium. The early loss of teeth at
the age of 4 results in decreased inflammation, and the
gingiva appears to be healthy. Similarly, the eruption of the
permanent teeth evokes a cycle of gingivitis and periodontitis
accompanied by subsequent premature exfoliation in the
early teenage years, resulting in alveolar bone loss and
a decrease in facial height. Subsequently, after a period
of tooth loss, the third molars erupt with no sign of
inflammation.13
Because of the reduced immunity to pathogens in PLS
patients, Actinobacillus actinomycetemcomitans (Aa) and
other anaerobic bacteria have been proven to play a major
role in the periodontitis. 6 An improvement in clinical
symptoms has been observed with synchronized elimination
of Aa from the gingival crevice and the use of a systemic
antibiotic. 3 The conventional treatment measures for
periodontal disease are usually unsuccessful in controlling
periodontal disease associated with the syndrome.14 Previous
studies have shown that at the active stage of periodontitis,
it is possible to arrest further periodontal destruction with
early treatment and preventive measures.15-17 These comprise
oral hygiene instruction, the use of mouthwashes, frequent
scaling and debridement, the use of planned multiple systemic
132
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Orthodontic treatments of papillon-lefevre
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Baidas LF
antibiotic regimens, periodontal surgery, and extraction of
hopeless teeth.18
Limited information is available in the literature regarding
combined treatment of orthodontic tooth movement and
periodontal treatment.15-17 To our knowledge, the cases
presented here are two of the few cases to be published about
combined periodontal-orthodontic treatment for patients
with PLS. The aim in presenting these cases is to demonstrate
the possibility of creating space for the permanent teeth to
erupt, and of stabilizing the occlusion in young PLS patients
until they reach full arch development under a strict
periodontal treatment protocol.
congenitally missing bilateral lower second premolars, a
large restoration in the lower left first molar, and a lack of
space for the permanent dentition. The patient complained
of pain in the lower left first molar during eating, and the
panoramic radiograph confirmed the presence of a periapical
lesion. Accordingly, the patient was referred for root canal
treatment and restoration (Figure 1).
Fig 1: Pretreatment clinical photographs and panoramic radiograph
CASE REPORTS
The two cases presented were treated in the periodontics
and orthodontic clinics, at the College of Dentistry, King
Saud University, Riyadh, Saudi Arabia. for clarification, the
cases are numbered as 1 and 2.
CASE 1
A 10-year-old male patient was referred for evaluation
of his missing teeth and periodontal condition. His family
history revealed consanguineous marriage of the parents.
He was the third child born to the family. His elder sisters
and younger brothers were free of apparent genetic defects,
but the youngest sister also had PLS. The patient's medical
history revealed that symptoms started at the age of 4 months
in the form of desquamation and erythema on the hands and
feet. Plaques with pustules and a purulent discharge were
observed when he started walking. Treatment, including
cleaning of the lesions and systemic antibiotics, was
undertaken at the Department of Dermatology at King Faisal
Specialist Hospital. Blood tests including a complete blood
count, blood chemistry profile, and liver function tests
returned normal results. A genetic test followed by fluorescent
Sanger sequencing of exons 3 to 7 of the cathepsin C gene
revealed a mutation. The diagnosis of PLS was confirmed
from the clinical symptoms and the genetic test. The dental
history revealed that the deciduous teeth had erupted at the
normal age (6 months), but the patient experienced inflamed
and swollen gingiva after eruption, pain with mastication,
and mobility followed by spontaneous loss of the teeth. At
the age of 3 years, the second primary molars were the only
deciduous teeth remaining without any root resorption.
At the time of referral to the orthodontic clinic, intraoral
examination revealed that the patient was in the early mixed
dentition stage, with mild gingival inflammation and bleeding
on probing, and no mobility was observed. The panoramic
radiograph revealed slight horizontal alveolar bone loss,
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133
A strict periodontal regime was investigated by the
periodontist. Anti-infective therapy was started as the initial
phase, consisting of oral hygiene instruction, supragingival
and subgingival scaling, and rinsing with 0.2% chlorohexidine
mouthwash twice daily. Subgingival plaque samples of the
periodontally affected teeth were taken for microbiological
investigation to detect the presence of Aa. Systemic
amoxicillin (25 mg twice/day) and metronidazole (250 mg
three times/day) were prescribed for 2 weeks. The
maintenance program was continued during the orthodontic
treatment, including oral hygiene instruction and oral
prophylaxis once every 4 to 6 weeks, depending on the oral
hygiene condition of the patient.17
At the age of 10 years a lower lingual arch and an upper
Nance appliance were used to maintain space for the
permanent teeth and prevent further movement of the posterior
teeth into the edentulous area. At the age of 13 years, the
patient presented with a class I malocclusion on a skeletal
class I pattern with a normal vertical relationship, a straight
profile with a symmetrical face, and competent lips. The
dental characteristics were a class I molar relationship, a
class II canine relationship, an overbite of 80%, and a 4 mm
overjet that indicated a class I malocclusion. The upper
incisors were in normal inclination on the basal bone, and
the lower incisors were slightly retroclined. The lower right
and left second premolars were congenitally missing, and
the lower right and left second deciduous molars were
retained. The lack of space for the upper and lower first
premolars was readily detected, with -5 mm of crowding in
Orthodontic treatments of papillon-lefevre
syndrome: Two case reports
Baidas LF
the lower dentition, and -3 mm of crowding in the upper
dentition (Figure 2).
Fig 2: Pretreatment cast photographs and panoramic
radiograph before start of orthodontic treatment
continuous archwire mechanics from molar to incisor using
0.016, 0.018, and 0.016 × 0.022 SS archwires. Crowding of
the upper arch would thereafter be resolved by subsequent
interdental stripping between the posterior teeth (Figure 3).
Fig 3: Clinical photographs and panoramic radiograph
after a year of treatment
ORTHODONTIC OBJECTIVES
AND MANAGEMENT
The primary objectives of treatment were to correct the
anterior deep bite, taking advantage of alveolar growth
changes in the premolar-molar area. The other objectives
included relieving crowding and creating space in the upper
and lower arches, maintaining the lower deciduous second
molars, achieving class I canine and molar relationships,
and last stabilizing the occlusion.
Preadjusted edgewise fixed orthodontic appliances (0.22"
slot Roth prescription) were bonded in the lower jaw, and
then the upper jaw. Orthodontic treatment was begun in only
one jaw with very light force because of periodontal condition.
The main problems in the lower jaw were moderate crowding,
deficient space for the lower first premolars, a retained
deciduous second molar, congenitally missing second
premolars, and overeruption and retroclination of the lower
incisors. From the periodontal viewpoint, the incisors, canines,
and first molars exhibited minimal or no bone loss, and had
a good prognosis because of their later eruption with the
concomitant periodontal treatment. Overbite reduction
was regarded as a major orthodontic treatment goal, so a
016 × .022 beta-titanium (TMA) utility arch was used to
open the deep bite and create an incisal stop. Intrusive forces
were kept at very low levels (approximately 10-15 gm).
Initial alignment and leveling were achieved with 0.016"
copper nickel titanium (Cu-NiTi) archwire, followed by
0.016, 0.018, and 016 × .022 stainless steel (SS) archwires.
The space for the lower first premolars was created through
interdental stripping and the use of a NiTi push-coil spring
between the canine and the deciduous second molars. In the
upper jaw, the main problem was mild crowding, and slight
overeruption of the upper incisors. Therefore, alignment and
leveling were completed with 0.016" Cu-NiTi wire, and
excessive eruption of the upper incisors was corrected with
134
The total treatment duration was 24 months. Before the
fixed appliances were debonded, a good functional occlusion
had been established. The lower left deciduous second molar
exfoliated because of root resorption. Hawley retainers were
used for retention; the upper Hawley retainer included a
biteplate to control vertical growth, while the lower Hawley
retainer had an acrylic tooth in the edentulous space. The
protocol for retention was full-time use of the retainers for
3-4 months, followed by night-time use for several years to
control the vertical overlap of incisors. However, the lower
Hawley retainer was to be used full time until he was ready
for replacement of the missing second premolars with
implants. The treatment aims were accomplished successfully.
Fig 4: Post-treatment clinical photographs and panoramic radiograph
The deep bite was corrected, and normal overjet and overbite
were achieved. The canine relationship was corrected to a
class I relationship, the Angle class I molar relationship was
maintained, and the midline of the upper and lower arches
was corrected. The panoramic radiograph showed minimal
bone loss in both jaws, and vertical bone loss at the distal
surface of the lower left first molars. The distal root of the
lower right deciduous molar was resorbed, but the tooth was
JPDA Vol. 30 No. 02 Apr-Jun 2021
Orthodontic treatments of papillon-lefevre
syndrome: Two case reports
Baidas LF
still stable with no mobility. Our aim was to retain the
deciduous tooth for as long as possible until it could be
replaced with an implant (Figure 4). The patient exhibited
Fig 5: Clinical picture of the foot and hands showing
hyperkeratosis of the palms and soles
hyperkeratosis of the palms of the hand and the soles of the
feet, but these skin lesions subsided with age (Figure 5).
Post-treatment evaluation after 3 years revealed some
relapse of the overbite because the patient was not cooperative
in wearing the retainers. An OPG revealed no bone loss
compared with the previous OPG after debonding. The space
of tooth #35 had decreased as a result of drifting of the lower
left first molar (Figure 6).
Accordingly, the patient was referred to the pediatric genetic
department for further investigation. The dental history
revealed that during the first year after eruption of the incisors,
the gingiva had become inflamed and swollen with a purulent
discharge in response to the pressure of mastication. The
deciduous teeth erupted with normal sequence and timing.
At the age of 4 years, all the deciduous teeth were extracted
under general anesthesia, aiming for complete recovery of
the gingiva to its normal healthy status. Taking into
consideration the clinical features, laboratory investigations,
and genetic testing, a diagnosis of PLS was confirmed by
the patient's pediatrician.
The same protocol for periodontal management as for
the previous patient was followed. The patient was
subsequently referred to the orthodontic clinic. Intraoral
examination revealed gingival inflammation, and no tooth
mobility. She was in the mixed dentition stage, with a deep
overbite (more than 50%) and a lack of space for the
permanent dentition. A panoramic radiograph revealed space
loss and mesial drifting of the first molars (Figure 7). At the
Fig 7: Pretreatment clinical photographs and panoramic radiograph
Fig 6: 3-year post-treatment clinical photographs
and panoramic radiograph
CASE 2
A female patient aged 8 years diagnosed with PLS was
referred to the periodontics clinic by her primary care
practitioner. She was the second child of consanguineous
unaffected parents. Her elder brother also had PLS, but the
other three siblings were free of apparent genetic defects.
The patient's medical history revealed that desquamation
and erythema of the palms and soles were observed at the
age of 4 months, with a gradual onset, followed by thickening
and fissuring of both the soles. These symptoms worsened
with age. The patient presented at the dermatology clinic at
the National Guard Hospital, Riyadh, Saudi Arabia, when
she was 2 years old with a chief complaint of palmoplantar
hyperkeratosis. All laboratory tests, including hematological
and liver function tests, were normal. Various therapies were
undertaken to treat the skin lesions with no improvement.
JPDA Vol. 30 No. 02 Apr-Jun 2021
135
age of 9 years space maintainers were used in the form of
a lower lingual arch and an upper Nance appliance to maintain
the space for the permanent teeth to erupt and to prevent
further movement of the posterior teeth into the edentulous
area. Regular checks and follow-ups were maintained every
3-6 months until the permanent teeth erupted.
At the age of 14 years, the patient presented with class
I malocclusion with a skeletal class I pattern and a normal
vertical relationship, with mild crowding in the upper arch
and moderate crowding in the lower arch, a Class I molar
and canine relationship, and a 2-mm lower midline shift to
the left side. The panoramic radiograph revealed generalized
horizontal bone loss, a vertical pocket distal to the lower
right and upper left first molars, and mesial drifting of the
lower Left first molars. She was in the permanent dentition
stage, with all teeth erupted except the lower second premolars
and third molars. Root dilaceration was present in many of
Orthodontic treatments of papillon-lefevre
syndrome: Two case reports
Baidas LF
Fig 8: Pretreatment cast photographs and panoramic radiograph
before start of orthodontic treatment
the teeth, but the dilaceration was more severe in the right
upper lateral and right lower lateral teeth as compared with
upper second premolars and upper left lateral incisor. The
lack of space for the lower second premolars was readily
detected, with -5 mm of crowding in the lower dentition,
Fig 9: Clinical picture of
the Face showing
hyperpigmentation
around the mouth, and
Palm-planter hyperkeratosis
of the hand and foot
lower second premolars. From the periodontal viewpoint,
the erupted teeth exhibited minimal bone loss, except for
the lower right and left first molars, which had distal vertical
pockets. Hence, the planned treatment goals were expanding
the arch and creating enough space for eruption of the
premolars, and stabilizing the occlusion. The spaces for the
lower first premolars were achieved with interdental stripping
and push coil springs (Figure 10). A class II elastic was used
to correct the occlusion on the left side, and anterior cross
elastics were applied to correct the lower midline. The OPG
Fig 10: Clinical photographs and panoramic radiograph
after a year of treatment
revealed no bone loss and no further increase in the distal
vertical pocket of the lower first molars.
The total treatment duration was 15 months. The treatment
goals were accomplished, and a good functional occlusion
was established. The treatment resulted in a normal overjet
and overbite, and class I canine and molar relationships. A
panoramic radiograph showed no or only minimal bone loss
(Figure 11). During the whole period of orthodontic treatment,
the patient's periodontal status was regularly evaluated for
bleeding on probing, attachment loss, and pocket depth.
Fig 11: Post-treatment Clinical photographs and
panoramic radiograph
and +3 mm of spacing in the upper dentition (Figure 8). The
patient exhibited hyperkeratosis of the palms and soles, and
dryness and around the mouth (Figure 9).
ORTHODONTIC OBJECTIVES
AND MANAGEMENT
The objectives of the treatment were to relieve the
crowding in the lower arch, create space for the permanent
teeth, achieve class I canine and molar relationships, close
spaces, and stabilize the occlusion. Preadjusted edgewise
fixed orthodontic appliances (0.22" slot Roth prescription)
were bonded in the lower jaw, and then the upper jaw. A
very light force was used throughout the orthodontic
treatment. Initial alignment and leveling were achieved with
a maxillary 0.016" Cu-NiTi archwire, followed by 0.016,
and 0.016 ×0.022 SS wire. The main problems in the lower
jaw were moderate crowding and deficient space for the
136
Fig 12: 3-year post-treatment panoramic radiograph
JPDA Vol. 30 No. 02 Apr-Jun 2021
Orthodontic treatments of papillon-lefevre
syndrome: Two case reports
Baidas LF
Retention was accomplished by using a fixed lingual
canine-to-canine retainer in the lower arch and a Hawley
retainer in the upper arch. A 3-year post-treatment OPG was
taken to document the stability of the periodontal condition,
and showed no further resorption of bone (Figure 12).
DISCUSSION
The etiology of the periodontal problems in PLS patients
is not clearly understood. The increased prevalence of
periodontal disease in PLS patients gives credence to the
hypothesis that an underlying immunological deficiency is
an important etiological factor causing periodontitis in PLS
patients.7 This results in a reduced host response against
plaque bacteria and increased susceptibility to infection.19
Aa and Gram-negative bacteria were detected in subgingival
plaque, and were found to play a significant role in the
etiology of PLS periodontitis. 6 Patients with affected
periodontal tissue are at high risk of further breakdown and
loss of teeth.7 Early diagnosis and proper management of
periodontal problems helps to minimize periodontal
deterioration and the undesirable sequelae of the syndrome.
Periodontal therapy includes mechanical debridement by
scaling and polishing, systemic antibiotics to eliminate the
pathogen reservoir, extraction of hopeless mobile teeth,
maintenance of good oral hygiene, and regular monitoring
and recall appointments.12,19
Eradication of subgingival Aa and maintenance of good
oral hygiene are key factors in in preserving permanent
teeth in young PLS patients.18,20 In our reported cases, the
therapeutic approaches for the primary dentition period
were different. In the first case, all the primary teeth except
the lower second molars exfoliated several months after
eruption. A combination of systemic amoxicillin and
metronidazole in addition to the maintenance of good oral
hygiene was followed; this has been successful in some
cases.5 Another therapeutic approach to PLS, which was
followed in the second reported case, was to eradicate the
pathogenic periodontal flora by extracting all the primary
teeth before eruption of the permanent teeth, combined
with systemic antibiotic treatment to create a safe
environment for eruption of the permanent teeth. The
edentulous period combined with meticulous oral hygiene
determines the treatment outcome.20
After the edentulous period, the permanent teeth erupt
without any guidance, and this can lead to loss of space,
crowding, and collapse of the dental arch.15 The cases
reported here have demonstrated the potential for successful
orthodontic treatment of PLS patients under a controlled
regime of periodontal treatment. Orthodontic treatment was
performed to expand the arch, create space for the normal
JPDA Vol. 30 No. 02 Apr-Jun 2021
137
eruption of the permanent teeth, and stabilize the occlusion.
This treatment helps patients to gain a normal facial
appearance rather than the collapsed appearance caused by
early loss of teeth.15,20
Orthodontic treatment in PLS patients with periodontal
disease poses a high risk of exacerbating periodontal
breakdown and tooth loss.13 In the literature, information
about orthodontic treatment in PLS patients is limited.
However, there are several reported cases in which
follow-up of well-planned orthodontic treatment combined
with a periodontal regimen resulted in successful
maintenance of a healthy dentition.15-17 In the present case
reports, the patients were in the late mixed dentition period
with mild to moderate crowding along with mild generalized
bone loss and a deep overbite. A deep overbite is traumatic
to gingival tissue, and could cause occlusal trauma to the
upper anterior teeth and loss of periodontal support.21,22
Light force and moderate orthodontic tooth movements of
up to 3 mm were used over the entire treatment period with
good maintenance of oral hygiene. The treatment resulted
in well-aligned arches with acceptable occlusion and no or
only minimal bone loss. The panoramic radiographs taken
at the end of treatment showed a stable periodontal condition
with no bone loss. Evaluation at 3 years post-treatment
revealed good stability with retention. In the first case,
there was some relapse of the overbite because the patient
was not cooperative in wearing the retainers, or because
vertical growth continued into the late teens. However, in
the second case, the result was stable.
These case reports showed the need to discuss the
possibility of orthodontic tooth movement in patients with
PLS. More research and case reports are needed to document
treatment in patients with PLS of varying severity levels.
However, the present case reports revealed that orthodontic
tooth movement is possible in patients with PLS within a
combined interdisciplinary treatment protocol.
CONCLUSION
The present case reports demonstrate that PLS patients
can undergo successful combined orthodontic treatment
and strict periodontal therapy to achieve moderate
orthodontic tooth movement. Such a combined
interdisciplinary treatment regimen could be crucial in
achieving functional and esthetically pleasing dentition in
patients with PLS.
ACKNOWLEDGEMENTS
The author appreciatively acknowledges Prof. Nahid
Ashri, Consultant Periodontist, Department of Periodontics
Orthodontic treatments of papillon-lefevre
syndrome: Two case reports
Baidas LF
and Community Dentistry, College of Dentistry, King Saud
University, Riyadh, for her valuable and competent support
and advice. We also thank Helen Jeays, BDSc AE, from
Edanz Group (https://en-author-services.edanzgroup.com/ac)
for editing a draft of this manuscript.
syndrome. J Pediatr Surg. 1996;31:955-56.
https://doi.org/10.1016/S0022-3468(96)90420-0
11. Hart TC, Hart PS, Bowden DW, Michalec MD, Callison SA, Walker
SJ, Zhang Y, Firatli E. Mutations of the cathepsin C gene are responsible
for Papillon-Lefevre syndrome. J Med Genet. 1999;36:881-87.
12. Hattab FN. Papillon-Lefèvre syndrome: from then until now.
Stomatological Dis Sci. 2019;3:1
https://doi.org/10.20517/2573-0002.2018.22
CONFLICT OF INTEREST
None to declare
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