|Year : 1992 | Volume
| Issue : 2 | Page : 113-115
H Hanumanthappa, KK Ganesh
Source of Support: None, Conflict of Interest: None
A case of Paplillon-Lefevere syndrome is reported in a 12-year old boy. He had palmo-plantar keratoderma extending on to the pressure points of the body, early loss of deciduous and permanent teeth with Periodontosis and recurrent pyogenic infection of skin.
Keywords: Papillon - Lefθvre syndrome, recurrent pyogenic infection
|How to cite this article:|
Hanumanthappa H, Ganesh K K. Papillon-lefevre syndrome. Indian J Dermatol Venereol Leprol 1992;58:113-5
| Introduction|| |
Papillon-Lefθvre syndrome, a rare disorder of skin and periodontal supporting tissues, was first described by Papillon and Lefθvre in 1924.  It is believed to be of autosomal recessive inheritance. Syndrome is constituted by palmo-plantar hyperkeratosis and periodontosis leading to early loss of deciduous and permanent teeth. It usually manifests between the age 1 to 4 years although earlier onset had also been recorded.  Hyperkeratosis is transgredient; i.e., lesions extended a little over the wrists, ankles, dorsa of the hands and feet, knees and elbows. Tendency to clear spontaneously at puberty has been reported.  Nail changes have been described.  Dural calcification, especially attachment of tentorium and choroid has been observed in 1 case.  The defect in T-lymphocytes could be the responsible factor for recurrent suppurative infection of skin. 6
| Case Report|| |
A 12-year-old boy born to a consanguineous married couple presented with hyperkeratotic lesions of palms & soles, extending on to the dorsa of the feet and hands, elbows and knees. Early loss of both deciduous and permanent teeth [Figure - 1] and recurrent pyogenic infection of the skin with scars [Figure - 2] started from 3rd month of life onwards. His milk teeth erupted normally. They were non carious but became loose and fell off one by one by the age of 5 years. But the permanent teeth were lost haphazardly leaving behind 3 canines, 2 incisors and 1 premolar. All these teeth were loosened. Painful swelling of the gums with foul smelling pus discharge from the pockets around the teeth was observed. Pyoderma lesions with multiple atrophic scars were seen in both the gluteal and thigh regions. Transverse grooves were present on the toe nails.
Roentgenographic examination of the teeth revealed absence of all the teeth except 2 incisors, 3 canines and 1 premolar. The roots of these teeth were incomplete with open apices and there was severe destruction of periodontal supporting tissues. Skull X-ray was normal. Skin biopsy showed irregular hyperkeratosis, marked acanthosis and a conspicuous perivascular infiltrate of lymphocytes and histiocytes.
| Comments|| |
The incidence of Papillon-Lefθvre syndrome is 1-4 per million people. Our patient had exhibited palmo-plantar hyperkeratosis and hyperkeratosis over pressure points of the body, periodontosis with early loss of permanent teeth and recurrent pyogenic infection of skin with grooving of toe nails. Such associations are rare manifestations of the syndrome. Similar case was reported by Velou et al  sub in which recurrent skin infection was absent.
| References|| |
|1.||Akira Ohkawara, Yusho Miura, Jun Ishikawa Sapporo. Papillon-Lefevre Syndrome. Arch Dermatol 1974; 109: 726-8. |
|2.||Carson E F. Papillon-Lefevre Syndrome. Arch Dermatol 1939; 40: 639. |
|3.||Ziprkowski L, Ramon Y, Brish M. Papillon-Lefevre Syndrome. Arch Dermatol 1963; 88: 207. [PUBMED] |
|4.||Haim S, Munk J. Keratosis palmo-plantaris Congenita with periodonosis, arachnodactyly and a peculiar deformity of the terminal phalanges. Br J Dermatol 1965; 77: 42-54. [PUBMED] |
|5.||In : Textbook of Dermatology, (Arthur Rook et al, eds), 4th edn. Oxford University Press, 1987; 1458. |
|6.||Velou A, Naik D N, Sardari Lal. PapillonLefevre Syndrome. Ind J Dermatol Venereol Leprol 1969; 4 : 163 - 5. |
[Figure - 1], [Figure - 2]