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Papillon‐Lefèvre Syndrome in Four Siblings Treated with Etretinate A Nine‐Year Evaluation

Identifieur interne : 005876 ( Istex/Checkpoint ); précédent : 005875; suivant : 005877

Papillon‐Lefèvre Syndrome in Four Siblings Treated with Etretinate A Nine‐Year Evaluation

Auteurs : Robert E. Kellum [Arabie saoudite]

Source :

RBID : ISTEX:3A990110DBFD646FA0D40990A5A357EE70CF8EC0

English descriptors

Abstract

Four siblings with Papillon‐Lefèvre syndrome (3 boys and 1 girl), aged 8 to 12 at time of first diagnosis in 1978 are reported. These four patients represent the second largest sibship reported in the literature, and the only familial cases treated with etretinate for 6 years with an additional 3 1/2 year follow‐up evaluation. No long‐term side effects of etretinate were found in the children. All four patients are the product of a second cousin marriage; all demonstrate the B5 locus on HLA typing.

Url:
DOI: 10.1111/j.1365-4362.1989.tb02539.x


Affiliations:


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ISTEX:3A990110DBFD646FA0D40990A5A357EE70CF8EC0

Le document en format XML

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<wicri:regionArea>From the Department of Medicine, King Faisal Specialist Hospital and Research Centre Riyadh</wicri:regionArea>
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<wicri:regionArea>Correspondence address: Address for correspondence: Robert E. Kellum, M.D., Department of Medicine, King Faisal Specialist Hospital and Research Centre, P. O. Box 3354, Riyadh 11211</wicri:regionArea>
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<title level="j" type="main">International Journal of Dermatology</title>
<title level="j" type="alt">INTERNATIONAL JOURNAL OF DERMATOLOGY</title>
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<idno type="eISSN">1365-4632</idno>
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<term>Average dose</term>
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<term>Bright erythema</term>
<term>Dorsal feet</term>
<term>Dorsal surfaces</term>
<term>Epiphyseal lines</term>
<term>Etretinate</term>
<term>Etretinate therapy</term>
<term>Familial cases</term>
<term>Fasting triglycerides</term>
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<term>Hyperkeratosis</term>
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<term>Inflammatory gingivai disease</term>
<term>Keratosis palmoplantaris</term>
<term>Keratotic plaques</term>
<term>King faisal specialist hospital</term>
<term>Last visit</term>
<term>Ligamentous calcification</term>
<term>Month intervals</term>
<term>Normal limits</term>
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<term>Periodontosis syndrome</term>
<term>Permanent teeth</term>
<term>Plantar</term>
<term>Plantar feet</term>
<term>Plantar hyperkeratosis</term>
<term>Pretibial areas</term>
<term>Research centre</term>
<term>Saudi arabia</term>
<term>Serum immunoglobulins</term>
<term>Serum protein electrophoresis</term>
<term>Serum triglycerides</term>
<term>Sibling</term>
<term>Skeletal survey</term>
<term>Syndrome</term>
<term>Triglyceride</term>
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<term>American nchs charts</term>
<term>August</term>
<term>Average dose</term>
<term>Baseline laboratory evaluation</term>
<term>Bright erythema</term>
<term>Dorsal feet</term>
<term>Dorsal surfaces</term>
<term>Epiphyseal lines</term>
<term>Etretinate</term>
<term>Etretinate therapy</term>
<term>Familial cases</term>
<term>Fasting triglycerides</term>
<term>First diagnosis</term>
<term>First time</term>
<term>Hair loss</term>
<term>Hyperkeratosis</term>
<term>Hyperkeratosis palmoplantaris</term>
<term>Hyperkeratotic</term>
<term>Inflammatory gingivai disease</term>
<term>Keratosis palmoplantaris</term>
<term>Keratotic plaques</term>
<term>King faisal specialist hospital</term>
<term>Last visit</term>
<term>Ligamentous calcification</term>
<term>Month intervals</term>
<term>Normal limits</term>
<term>November</term>
<term>Periodontosis syndrome</term>
<term>Permanent teeth</term>
<term>Plantar</term>
<term>Plantar feet</term>
<term>Plantar hyperkeratosis</term>
<term>Pretibial areas</term>
<term>Research centre</term>
<term>Saudi arabia</term>
<term>Serum immunoglobulins</term>
<term>Serum protein electrophoresis</term>
<term>Serum triglycerides</term>
<term>Sibling</term>
<term>Skeletal survey</term>
<term>Syndrome</term>
<term>Triglyceride</term>
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<div type="abstract" xml:lang="en">Four siblings with Papillon‐Lefèvre syndrome (3 boys and 1 girl), aged 8 to 12 at time of first diagnosis in 1978 are reported. These four patients represent the second largest sibship reported in the literature, and the only familial cases treated with etretinate for 6 years with an additional 3 1/2 year follow‐up evaluation. No long‐term side effects of etretinate were found in the children. All four patients are the product of a second cousin marriage; all demonstrate the B5 locus on HLA typing.</div>
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