Indexed with PubMed and Science Citation Index (E) 
Users online: 1338 
     Home | Feedback | Login 
About Current Issue Archive Ahead of print Search Instructions Online Submission Subscribe What's New Contact  
  Navigate here 
   Next article
   Previous article 
   Table of Contents
 Resource links
   Similar in PUBMED
    Search Pubmed for
    Search in Google Scholar for
   Article in PDF (233 KB)
   Citation Manager
   Access Statistics
   Reader Comments
   Email Alert *
   Add to My List *
* Registration required (free)  

  In this article
   Article Figures

 Article Access Statistics
    PDF Downloaded309    
    Comments [Add]    
    Cited by others 3    

Recommend this journal


Year : 2008  |  Volume : 74  |  Issue : 1  |  Page : 71-73

Acitretin for Papillon-Lefevre syndrome in a five-year-old girl

1 Department of Dermatology, Mustafa Kemal University, Faculty of Medicine, Antakya, Turkey
2 Department of Pediatrics, Mustafa Kemal University, Faculty of Medicine, Antakya, Turkey
3 Department of Dermatology, Antakya State Hospital, Antakya, Turkey

Correspondence Address:
Didem Didar Balci
Department of Dermatology, Mustafa Kemal University, Faculty of Medicine, 31100 Antakya
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0378-6323.38423

Rights and Permissions

How to cite this article:
Balci DD, Serarslan G, Sangun O, Homan S. Acitretin for Papillon-Lefevre syndrome in a five-year-old girl. Indian J Dermatol Venereol Leprol 2008;74:71-3

How to cite this URL:
Balci DD, Serarslan G, Sangun O, Homan S. Acitretin for Papillon-Lefevre syndrome in a five-year-old girl. Indian J Dermatol Venereol Leprol [serial online] 2008 [cited 2020 Jun 2];74:71-3. Available from: http://www.ijdvl.com/text.asp?2008/74/1/71/38423


Papillon-Lefevre Syndrome (PLS) is a rare genetic disorder with an autosomal recessive mode of inheritance and is characterized by symmetrical transgradient palmoplantar keratoderma and severe periodontal disease. Other features may include tendency to frequent pyogenic infections of the skin and internal organs, and calcification of the dura, particularly the tentorium and falx cerebri. The disease prevalence is one to four persons per million. Both sexes are equally affected. Consanguinity has been observed in one-third of the cases described. [1],[2] A few clinicians have previously reported that acitretin treatment is effective. [2],[3],[4],[5]

We present experience of acitretin therapy in a case of PLS, who had an excellent response in palmoplantar skin lesions within 2 weeks.

A five-year-old girl presented with palmoplantar lesions that started at the age of 5 months. She also had periodontal problems with loss of multiple incisor and premolar teeth. The patient suffered from teeth problems for seven to 8 months. The parents were first cousins. She had no family history of similar disease. She had no response to previous therapy with topical corticoid, urea and salicylic acid ointments. On dermatologic examination, she had bilateral well-demarcated hyperkeratotic plaques with fissures on the palmoplantar region [Figure - 1]. Oral examination revealed red and swollen gingivae associated with premature loss of deciduous teeth. Her weight was 15 kg with normal growth parameters. A pediatrician examined the patient, found no physical abnormality other than the above-mentioned. The patient was referred to a dentist who advised good dental care and bimonthly dental examination. Routine blood count, liver and kidney function tests, cholesterol, triglycerides and urinalyses were within normal limits. No abnormality was found on abdominal ultrasound examination and cranial tomography. X-ray of left hand was compatible with the chronological age of the patient. A skin biopsy specimen from the plantar region revealed hyperkeratosis, irregular acanthosis, dilated vessels within the papillary dermis and perivascular lymphocytic infiltrates.

A diagnosis of PLS was made after dermatological, dental and histopathological examinations. The patient was given acitretin 10 mg PO every day, omitting the third day. The lesions improved with a marked reduction in the hyperkeratosis 2 weeks after the treatment [Figure - 2]. The treatment was maintained for 5 months. The patient's skin remained almost lesion-free following the therapy. Her gingivae also showed remarkable improvement. Her unstable deciduous teeth were extracted and replaced by a dental prosthesis. The biochemical tests were performed every month. X-ray of the left hand was repeated at the end of the treatment. Neither skeletal nor biochemical side-effects were seen. The acitretin therapy was discontinued, followed by the rapid recurrence of the palmoplantar keratoderma within 2 weeks. Intermittent therapy is planned during exacerbations of the disease, especially in the winter months.

A multidisciplinary approach including the dermatologist, pediatrician and dentist is important for the therapeutic management of PLS. Different therapeutic options have been used for the management of the PLS-associated palmoplantar keratoderma. Topical keratolytics containing salicylic acid and urea have been used. Especially in winter, palmoplantar hyperkeratosis can worsen with painful fissures limiting routine activities and necessitating systemic treatment. [3] Extraction of the primary teeth combined with oral antibiotics and professional oral hygiene care are measures to improve periodontitis. [6],[7] On the other hand, these therapies do not frequently achieve protection of permanent teeth. [7]

A number of authors used systemic acitretin in the treatment of palmoplantar keratoderma and, reported that this therapy is effective at a dose of ranging between 0.4 and 1 mg/kg/day. [2],[3],[4] The authors noted significant improvement after four to 6 weeks of this therapy. The dose of acitretin was gradually tapered in most of these reports. Lee et al., also reported improvement of periodontal disease after 12 month-therapy with acitretin. [4] Nazzaro et al., reported satisfactory improving of palmoplantar keratoderma and periodontal disease in all three patients which was continued for 16 months of therapy. [5]

According to the above mentioned reports, palmoplantar keratoderma usually recovers rapidly whereas periodontal disease tends to improve later. In our case, the palmoplantar keratoderma had markedly improved in the second week of the therapy. The patient's skin remained almost lesion-free during the 5 month-therapy. Her gingivae also showed remarkable improvement. However, a rapid recurrence of the palmoplantar keratoderma was observed when the acitretin therapy was discontinued.

Many authors have suggested that use of oral retinoids for prolonged periods is useful to prevent loss of permanent teeth in children with PLS. [4],[5] The safety of oral retinoids in children remains controversial due to their side-effects on skeletal development. However, a review of the use of acitretin (mean dosage of 0.47 mg/kg) in 46 children for a cumulative period of 472 months revealed that it is a safe and effective treatment in children with keratinization disorders. [8]

In conclusion, the use of low-dose of acitretin in treatment of PLS-associated palmoplantar keratoderma is extremely useful but not curative. The current report suggests that low dosage of acitretin is safe and effective in the treatment of PLS.

  References Top

1.Haneke E. The Papillon-Lefιvre syndrome: Keratosis palmoplantaris with periodontopathy: Report of a case and review of cases in the literature. Hum Genet 1979;51:1-35.  Back to cited text no. 1    
2.Khandpur S, Reddy BS. Papillon-Lefιvre syndrome with pyogenic hepatic abscess: A rare association. Pediatr Dermatol 2001;18:45-7.  Back to cited text no. 2    
3.Al-Khenaizan S. Papillon-Lefιvre syndrome: The response to acitretin. Int J Dermatol 2002;41:938-41.  Back to cited text no. 3    
4.Lee MR, Wong LF, Fischer GO. Papillon-Lefιvre syndrome treated with acitretin. Australas J Dermatol 2005;46:199-201.  Back to cited text no. 4    
5.Nazzaro V, Blanchet-Bardon C, Mimoz C, Revuz J, Puissant A. Papillon-Lefιvre syndrome: Ultrastructural study and successful treatment with acitretin. Arch Dermatol 1988;124:533-9.  Back to cited text no. 5    
6.Preus HR. Treatment of rapidly destructive periodontitis in Papillon-Lefιvre syndrome: Laboratory and clinical observations. J Clin Periodontol 1988;15:639-43.  Back to cited text no. 6    
7.Kim JB, Morita M, Kusumoto M, Watanabe T, Takagi S, Nishijima K. Preservation of permanent teeth in a patient with Papillon-Lefιvre syndrome by professional tooth-cleaning. ASDC J Dent Child 1997;64:222-6.  Back to cited text no. 7    
8.Lacour M, Mehta-Nikhar B, Atherton DJ, Harper JI. An appraisal of acitretin therapy in children with inherited disorders of keratinization. Br J Dermatol 1996;134:1023-9.  Back to cited text no. 8    


  [Figure - 1], [Figure - 2]

This article has been cited by
1 Papillon-Lèfevre syndrome with palmoplantar keratoderma and periodontitis, a rare cause of pyrexia of unknown origin: a case report
Somia Iqtadar,Sami Ullah Mumtaz,Sajid Abaidullah
Journal of Medical Case Reports. 2015; 9(1)
[Pubmed] | [DOI]
2 Low-dose acitretin in Papillon-Lefèvre syndrome: treatment and 1-year follow-up
Nilendu Sarma,Chiranjit Ghosh,Sudipta Kar,Badruddin Ahmed Bazmi
Dermatologic Therapy. 2014; : n/a
[Pubmed] | [DOI]
3 Two cases of papillon-lefévre syndrome
Cho, H.-H., Kim, S.-H., Seo, S.-H., Jung, D.-S., Ko, H.-C., Kim, M.-B., Kwon, K.-S.
Korean Journal of Dermatology. 2009; 47(4): 472-478


Print this article  Email this article
Previous article Next article


Online since 15th March '04
Published by Wolters Kluwer - Medknow