Papillon‐Lefèvre Syndrome in Four Siblings Treated with Etretinate A Nine‐Year Evaluation
Identifieur interne : 005876 ( Istex/Checkpoint ); précédent : 005875; suivant : 005877Papillon‐Lefèvre Syndrome in Four Siblings Treated with Etretinate A Nine‐Year Evaluation
Auteurs : Robert E. Kellum [Arabie saoudite]Source :
- International Journal of Dermatology [ 0011-9059 ] ; 1989-11.
English descriptors
- KwdEn :
- American nchs charts, August, Average dose, Baseline laboratory evaluation, Bright erythema, Dorsal feet, Dorsal surfaces, Epiphyseal lines, Etretinate, Etretinate therapy, Familial cases, Fasting triglycerides, First diagnosis, First time, Hair loss, Hyperkeratosis, Hyperkeratosis palmoplantaris, Hyperkeratotic, Inflammatory gingivai disease, Keratosis palmoplantaris, Keratotic plaques, King faisal specialist hospital, Last visit, Ligamentous calcification, Month intervals, Normal limits, November, Periodontosis syndrome, Permanent teeth, Plantar, Plantar feet, Plantar hyperkeratosis, Pretibial areas, Research centre, Saudi arabia, Serum immunoglobulins, Serum protein electrophoresis, Serum triglycerides, Sibling, Skeletal survey, Syndrome, Triglyceride.
- Teeft :
- American nchs charts, August, Average dose, Baseline laboratory evaluation, Bright erythema, Dorsal feet, Dorsal surfaces, Epiphyseal lines, Etretinate, Etretinate therapy, Familial cases, Fasting triglycerides, First diagnosis, First time, Hair loss, Hyperkeratosis, Hyperkeratosis palmoplantaris, Hyperkeratotic, Inflammatory gingivai disease, Keratosis palmoplantaris, Keratotic plaques, King faisal specialist hospital, Last visit, Ligamentous calcification, Month intervals, Normal limits, November, Periodontosis syndrome, Permanent teeth, Plantar, Plantar feet, Plantar hyperkeratosis, Pretibial areas, Research centre, Saudi arabia, Serum immunoglobulins, Serum protein electrophoresis, Serum triglycerides, Sibling, Skeletal survey, Syndrome, Triglyceride.
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:3A990110DBFD646FA0D40990A5A357EE70CF8EC0Le document en format XML
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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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<idno type="eISSN">1365-4632</idno>
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<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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<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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<front><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>
</front>
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