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Year : 1998  |  Volume : 64  |  Issue : 6  |  Page : 303-304

Papillon - lefevre syndrome with mental retardation

Correspondence Address:
Vijay Gandhi

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Source of Support: None, Conflict of Interest: None

PMID: 20921806

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A 9-year old boy, born of consanguineous parents, presented with keratoderma of palms and soles associated with severe enamel hypoplasia and mental retardation. A clinical diagnosis of Papillon - Lefevre was supported by histopathology.

Keywords: Keratoderma , Papillon-Lefevre syndrome, Mental retardation, Enamel hypoplasia

How to cite this article:
Gandhi V, Singal A, Mohanty S, Bhattacha. Papillon - lefevre syndrome with mental retardation. Indian J Dermatol Venereol Leprol 1998;64:303-4

How to cite this URL:
Gandhi V, Singal A, Mohanty S, Bhattacha. Papillon - lefevre syndrome with mental retardation. Indian J Dermatol Venereol Leprol [serial online] 1998 [cited 2020 Jun 2];64:303-4. Available from: http://www.ijdvl.com/text.asp?1998/64/6/303/4735

  Introduction Top

Papillon-Lefevre syndrome is a rare disorder of keratinisation characterised by keratoderma of palms and soles, associated with periodontosis and tendency to pyogenic skin infections.[1] Mental retardation, ectopic intracranial calcifications and acro-osteolysis are rarely described associations.[2][3][4][5] We report here, a case of this rare syndrome with associated mental retardation.

  Case Report Top

A 9-year-old boy presented with gradually progressive keratoderma of palms and soles with severe fissuring. The thickening of soles had first been noticed by the parents when he was 2 year old, followed by involvement of palms. The parents also complained of impaired mental functions in the child and this had prevented him from attending school. Examination revealed an intellectually impaired boy who could follow verbal commands with difficulty. Cutaneous examination revealed thickening of palms and soles with fissuring encroaching to the dorsa of toes and feet, with mild localised thickening of elbows and knees. In addition, the patient had severe enamel hypoplasia with upper central incisors showing serrated and irregular surfaces [Figure - 1]. X-ray of maxilla (Water's view) and central incisors revealed no abnormality of the roots, and x-ray skull did not show any evidence of intracranial calcification. The child was born to consanguineous parents. No other family member had similar cutaneous manifestations.

A skin biopsy from sole showed hyperkeratosis and acanthosis, features consistent with diagnosis of keratoderma.

  Discussion Top

Palmoplantar keratoderma is a group of inherited dermatoses characterised by thickening of palms and soles, associated with other systemic abnormalities. The most satisfying criterion for classifying the group is to separate it into two distinct groups-a dominant and a recessive one. Further subclassification must consider the morphological aspects and other associations. Histopathology is essentially similar in all subgroups. Papillon-Lefevre syndrome is an autosomal recessive type characterised by association of periodontosis resulting severe gingivitis and recurrent pyogenic infections of the skin. Our patient, in addition, to severe enamel hypoplasia and gingivitis also had mental retardation which is one of the uncommon associations described in literature. Local therapy in the form of emollients with keratolytic was instituted with satisfactory results.

  References Top

1.Griffiths WAD, Leigh IM, Marks R. Disorders of keratinisation, in: Champion RH, Burton JL, Ebling FJG (eds), Textbook of Dermatology, Oxford Blackwell,1992, p1325.  Back to cited text no. 1    
2.Giorgi V. De, Martini L, Prignano F, et al. Papillon-Lefevre syndrome in two sisters. J Eur Acad Dermatol Venereol 1996;6:57-61..  Back to cited text no. 2    
3.Trattner A, David M, Sandbank M, et al. Papillon-Lefevre syndrome with acroosteolysis. J Am Acad Dermatol 1991;24:835-838..  Back to cited text no. 3    
4.Haini S, Munk J. Keratosis palmoplantaris congenita with periodontosis, arachnodactyly and a peculiar deformity of the terminal phalanges. Br J Dermatol 1965;77:42-54.  Back to cited text no. 4    
5.Nazzaro V, Blancheti-Bardon C, Mimoz C, et al. Papillon-Lefevre syndrome. Arch Dermatol 1988;124:533-539.  Back to cited text no. 5    


[Figure - 1]


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