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Year : 1993  |  Volume : 59  |  Issue : 1  |  Page : 11-14

Papillon-lefevre syndrome

Correspondence Address:
PVS Prasad

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Seven cases of Papillon-Lefevre syndrome from 6 families were studied. Their ages ranged from 3 to 25 years. Falling of deciduous teeth was noticed between 2 to 7 years. Five patients developed palmoplanatar keratoderma at 3 months of age. Two patients showed punctate lesions also. Only 1 patient was born to consanguinous parents.

Keywords: Papillon Lefθvre syndrome

How to cite this article:
Prasad P, Padmavathy L, Anandaraj A. Papillon-lefevre syndrome. Indian J Dermatol Venereol Leprol 1993;59:11-4

How to cite this URL:
Prasad P, Padmavathy L, Anandaraj A. Papillon-lefevre syndrome. Indian J Dermatol Venereol Leprol [serial online] 1993 [cited 2020 May 28];59:11-4. Available from: http://www.ijdvl.com/text.asp?1993/59/1/11/3867

  Introduction Top

Papillon and Lefθvre described 'the association of palmoplantar keratoderma (PPK) with periodontosis in 1924. [1] Other features of the syndrome are recurrent pyogenic skin infections and calcification of falx cerebri. [2],[3],[4] The dental features include frequent periodontal breakdown in both deciduous and permanent dentitions. Loosening of tooth and shedding occurs due to loss of alveolar bone. Gingiva becomes inflammed and bleed easily. Deciduous teeth are lost by the age of 5 years and permanent teeth by 15 years. More than 120 cases have been reported in the world literature upto 1988, [5],[6] of which about 10 are from our country. PPK may be punctate, striate, or diffuse. This starts between first and fourth years of life. [7] Keratoderma involves palms, soles, back, heel, external malleolus, tibial tuberosities, elbows, and dorsae of hands and feet. Maximum degree of hyperkeratosis coincides with active periodontal disease. [6] There may be occasional association with hypertension, hyperglycemia, systolic murmurs, retardation of skeletal maturation, osteoporosis, and hyper-thyroidism. [8] Etretinate, isotretinoin, and acitretin have all been successful in improving the cutaneous eruption, lessening the gingival inflammation, and in saving the teeth in severe cases. [9]

  Case Reports Top

Seven patients with Papillon-Lefθvre syndrome were seen. The clinical data of each patient was recorded in a proforma.

Dental examination was done in 5 patients and dental X-rays were taken wherever feasible.

Among 7 patients, there were 6 males and 1 female[Table - 1].

Their ages ranged from 3 to 25 years. Five patients developed rough palmar and plantar skin 3 months after birth, the other 2 at 6 months and 1 year respectively. The teeth which erupted normally, started loosening and falling from 2 1/2 to 7 years of age. One male patient who was seen at the age of 18 years was born to consanguineous parents [Figure - 1]. All other patients including 2 siblings were children of nonconsanguineous parents. Two male patients were affected with recurrent pyoderma on the lower limbs since childhood. Hyperhidrosis was noticed in 2 patients. None of our patients had any systemic abnormality. Dental examination done in 5 patients revealed less number of teeth than their age, and most of the teeth present were in grade I or grade II mobility. One adult patient seen at the age of 17 years had lost most of his permanent teeth. Two other adult patients who were examined at their residence also lost most of their permanent teeth. Dental X-rays revealed the features of missing teeth and loss of alveolar bone support. Routine and additional investigations like serum calcium, phosphorous, and alkaline phosphatase levels did not reveal any abnormality.

Case no.1 at the age of 3 1 /2 years showed premature exfoliation of tooth numbers 54 & 65. The other teeth were in grade I to grade 11 mobility. In the second patient 4 upper and 4 lower incisors, and 4 first molars should have erupted along with deciduous canine, first and second molars. All these were missing along with permanent incisors, permanent first molars, and all deciduous canines. The other teeth which were present were mobile. the third patient had no teeth in the right mandibular region which were extracted because of grade III mobility. In the fourth patient all deciduous canines and first molars which should have been present were not seen In the sixth patient a full complement of permanent teeth .excluding the third molars should have been present whereas he had only 4 first molars which were also in grade I mobility. One patient showed early loss of tooth by the age of 2 1/2 years which was very early for his age group.

Dental X-rays showed complete resorption of alveolar bone giving the teeth "floating-in-air" appearance [Figure - 2]. [6] Our patients did not show dural calcification or any other rarer associations like hypertension or hyperglycemia.

  Comments Top

PPK has been reported to occur between 1-5 years of life whereas majority of our patients (5 out of 7) developed this at 3 months of age. [7] The skin lesions of PPK might extend beyond the palms and soles which was seen in 2 of our patients. [5]

Papillon-Lefθvre syndrome is probably inherited as an autosomal recessive disease but only 1 patient in our study (case no.6) was born to consanguineous parents. Two of our patients had history of recurrent pyoderma on the legs since childhood. This phenomenon is usually seen due to disordered leukocyte function or decreased neutrophil phagocytosis. [3][4],[5]

The interesting feature noticed was that 6 out of 7 patients belonged to one village. Detailed epidemiological study could not pinpoint any particular reason for this.

  References Top

1.Ohkawara A,Miura Y, Sapporo JI. Papillon-Lefevre syndrome. Arch Dermatol 1974; 109: 726-8  Back to cited text no. 1    
2.Griffiths WAD, Leigh IM, Marks R. Disorders of keratinization. In: Textbook of Dermatology (Champion RH, Burton JL, Ebling FJG, eds), 5th edn. London: Blackwell Scientific 1992; 1377-9.  Back to cited text no. 2    
3.Velou A, Naik DN, Lal S. Papillon-Lefevre syndrome: Ind J Dermatol Venereol Leprol 1969; 4: 163-5.  Back to cited text no. 3    
4.Bregman R,Fried man-Birnbaum R. Papillon-Lef6vre syndrome: a study of the long term clinical course of recurrent pyogenic infections and the effects of etretinate treatment. Br J Dermatol 1988; 119: 731-6.  Back to cited text no. 4    
5.Williams ML. Ichthyosis and disorders of cornification. In : Pediatric Dermatology (Schachner LA, Hansen RC, eds), 1st edn. New York: Churchill Livingstone, 1988; 418-9.  Back to cited text no. 5    
6.Hebeil AA, Esterly NB. Mucous membrane disorders. In: Pediatric Dermatology (Schachner LA, Hansen RC, eds), 1st Edn. New York: Churchill Livingstone, 1988; 482-3.  Back to cited text no. 6    
7.Carson EF. Papillon-Lefevre syndrome. Arch Dermatol 1939; 40: 639.  Back to cited text no. 7    
8.Zeligman I. Palmar and panter hyperkertosis. In: Clinical Dermatology (Dermis DJ,Dobson RL, MC Guire J Harper, eds). Hangerstown: Harper and Row, 1980; 1-31.  Back to cited text no. 8  [PUBMED]  
9.Gharpuray MB, Patil DJ, Tolat SN, et al. Papillon-Lef6vre syndrome. Ind J Dermatol Venereal Leprol 1986; 52; 43-5.  Back to cited text no. 9    


[Figure - 1], [Figure - 2]


[Table - 1]

This article has been cited by
1 Papillon - Lefevre syndrome in two brothers
Rathi, S.
Indian Journal of Dermatology, Venereology and Leprology. 2002; 68(3): 155-156


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