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LETTER TO EDITOR
Year : 1994  |  Volume : 60  |  Issue : 3  |  Page : 177-178

Papillon-lefevre syndrome



Correspondence Address:
VV Rao Narsimha


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How to cite this article:
Narsimha VR. Papillon-lefevre syndrome. Indian J Dermatol Venereol Leprol 1994;60:177-8

How to cite this URL:
Narsimha VR. Papillon-lefevre syndrome. Indian J Dermatol Venereol Leprol [serial online] 1994 [cited 2019 Aug 20];60:177-8. Available from: http://www.ijdvl.com/text.asp?1994/60/3/177/4035


To the Editor,

In 2 cases of Papilion-Lefevre syndrome slightly different morphological features were seen by me. Both the patients are brothers; 1 is 6-years-old and other is 6-months-old. Parents are not consanguineous. Psoriasiform lesions are present not only on classical sites but on many other areas over the body in the elder child. Because of very rarity (only 10 cases are reported upto 1988 from our country).' These cases are discussed here.

A 6-year-old boy and his 6 months brother were brought to skin department for thickness and scaliness of palms and soles. On examination, palmoplantar hyperkeratosis with erythema and scales are noticed in both children. In elder child - psoriasiform scales are present on palms, soles, both knees, elbows, over tibia and malleoli. Within 1 month after coming to us patient developed psoriasiform plaques in many areas on the body. All deciduous teeth are lost except deciduous canine on right side of lower jaw, and permanent Ist molar on left side of lower jaw. X-ray mandible showed permanent tooth bud of 1st and 2nd premolar. No calcifiction of falx cerebre was seen.

Papillon-Lefevre syndrome is an autosomal recessive disorder of keratinization characterised by erythematous scales, thickness of palms and soles, psoriasis like lesions on elbow, knees, weakness of periodontial ligaments and teeth loss with calcification of falx cerebri. Localized disorders of keratinization such as mal de maleda, Unna Thost, Papillon - Lefevre syndrome may have strong relation with psoriasis.

Not only the present thinking of disordered leucocyte function, disordered gingival fibroflast, and cementoblast function in P-L syndrome, some other etiological factor such as Zinc deficiency in Acrodermatitis enteropathica may come into our notice in future. In this contest, response of psoriasis to linolenic acid may be thought of. In my patients cutaneous lesions responded well with external application of MF3 ointment (moisturizing ointment) retinoic acid, ointment, oral beta carotene, oral alfalin capsules (Linolic + Linolenic acid) massage of sunflower oil before bath.




 

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