papillon-lefevre syndrome: report of two cases


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    M. K. Hamdani, M.D.

    Basrah, Iraq

    Papillon-Lefevre syndrome is a disease ofchildhood characterized by hyperkeratosisof palm and sole combined with periodon-tosis; it was described by Papillon andLefevre in 1924. They described the condi-tions of two children (boy and girl) underthe title of familial palmar and plantarkeratoderma with abnormalities of teeth.iSince then a few cases have been recordedin France and other countries.2.3,4

    The erythema and keratosis of the feetand hand usually appear from one to fiveyears of age. The hyperkeratosis is not

    Consultant dermatologist, Teaching Hospital;lecturer in dermatology, Basrah Medical College,Basrah, Iraq.

    Accepted for publication Nov. 15, 1972.

    limited and may extend to the dorsum ofthe feet and hands. Although this syn-drome resembles meleda disease it differsby the presence of osseous lesions; bothare hereditary, autosomal recessive inorigin. The keratosis is more severe inwinter. In summer sweating may cause anunpleasant odor; the hair and nails arenormal.

    The deciduous teeth erupt normally butshortly afterwards they become loose be-cause of dystrophic changes of the alveolarbone with formation of periodontal pocketsand suppuration.5 The permanent teetherupt normally, but thereafter alveolardystrophies appear and the teeth becomeloose and fall out; the gum and oral mucosa

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    rig. 1 Pedigreeof the two familiesof the patients.

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    Fig. 2Patient 1, lost teeth.

    arc normal. Some cases have been reportedto be cured at puberty after expulsion ofthe teeth.6 In others the skin conditionimproves at that stage.

    The skeletal bones are normal. One ofmy patients showed obvious retardation ofsomatic and skeletal development. Con-sanguinity of the parents was present inmy two patients (Fig. 1).

    Arachnodactyly with claw-like terminalphalanges also has been described in onefamily.''

    Case ReportsCase 1. A 10-year-old Iraqiii boy com-

    plained of pahnoplanCar keratosis. Hislesion had appeared when he was 4, asscaly erythematous patches on the palmand sole. At examination the palm andsole were hyperkeratosed; slight erythemaand scaliness were seen on the dorsa of thefeet and hands. The boy's general condi-tion was normal, although he was short forhis age; bone growth appeared normal.Some of his permanent teeth had been lost(Fig. 2). X-rays of the gums showed erosionof the alveoli. Laboratory tests were withinnormal limits. Histopathologic examina-

    l ig . 3Gum o Patient 2.

    tion of the skin of the palm showed hyper-keratosis, canthosis and parakeratosis withperivascular cell infiltration.

    It was learned that his parents werecousins (Fig. 1).

    Fig. 4Hyperkeratosis o the sole in Patient 2.


    Fig. 5Patient 1, hyperkeratosis of the palm.

    Case 2. A 14-year-old Iraqui boy wasl-eferred to us from the dental unit; he hadlost all but four wisdom teeth of hispermanent teeth (Fig. 3). At 18 monthshe had begun to lose his deciduous teeth.When he was 5 the permanent teetherupted normally, but became loose andbegan to fall out. On examination the boywas healthy except for palmoplantar hyper-keratosis more marked on the sole (Figs.4, 5). Numerous fissures on the soles madewalking painful. Slight keratosis withscaliness was present on the knee and dorsaof the feet and hands. Skin biopsy fromthe foot showed hyperkeratosis with someinfiltrating cells. The mouth mucosa, therest of the skin and nails and hair werenormal.

    His parents were cousins; the boy's twosisters were normal.

    SummaryThe author reports two cases of Papillon-

    Lefevre syndrome. The two boys affectedhad palmoplantar hyperkatosis, periodon-

    Fig. 6X-ray of the jaw and mandible of Patient 2.

    tosis and suppuration of the alveoli. Mostof one boy's permanent teeth fell out, andall btit four molars have been lost by theother.

    References1. Papillon P, Lcfevre: Keratodeima palmairc ct

    plantaiie esymetric familiale et dialteiationdentaire. Bull Soc Fiance Dermatol Syph 31;82,1924

    2. Botaille R, Dupeiiat: La dentalion dans leskeratodermies palmo plantaire congcntales.Bull Soc France Derraatol Syph 59:121, 1953

    3. Bellengere E: Alveolyse infantile chute pre-coce des dents temporaiics ct peimanentcs etkeratose palmoplantaire: Un syndrome recessif.Thesis. Paris, 1954.

    4. Jansen LIT, Dekkcr C: Hyperkeratosis palino-plantaris with periodontosis (Papillon-Lefevre).Dermatologlca 113:207, 1956 ;

    5. Ziprkowski L, Ramon Y, Brisli M: Hyper-keratosis palmo plantaris with periodontosis(Papillon-Lefevre). Arch Dermalol 88:207, 19G3

    6. Greither A: Keratosis palmo plantaris mitpcriodontopathie (Papillon-Lefevre). Derma-tologica 119:248, 1959

    7. Haim S, Munk G: Palmoplantar keratosiswith periodontosis (Papillon-Lefevre Syn-drome). Br J Dermatol 77:42, 1965 :