Persistent lymphoedema in Morbihan disease: formation of perilymphatic epithelioid cell granulomas as a possible pathogenesis.
Identifieur interne : 002E36 ( Ncbi/Checkpoint ); précédent : 002E35; suivant : 002E37Persistent lymphoedema in Morbihan disease: formation of perilymphatic epithelioid cell granulomas as a possible pathogenesis.
Auteurs : T. Nagasaka [Japon] ; T. Koyama ; K. Matsumura ; K R ChenSource :
- Clinical and experimental dermatology [ 1365-2230 ] ; 2008.
Descripteurs français
- KwdFr :
- MESH :
- immunologie : Cellules épithélioïdes, Dermatoses faciales, Granulome, Histiocytes, Lymphoedème, Peau, Rosacée, Vaisseaux lymphatiques.
- Humains, Mâle, Sujet âgé, Syndrome.
English descriptors
- KwdEn :
- MESH :
- immunology : Epithelioid Cells, Facial Dermatoses, Granuloma, Histiocytes, Lymphatic Vessels, Lymphedema, Rosacea, Skin.
- Aged, Humans, Male, Syndrome.
Abstract
Morbihan disease is a rare complication of rosacea, characterized by persistent lymphoedema on the upper half of the face, occurring during the chronic clinical course of rosacea. This refractory condition has been also designated as 'rosacea lymphoedema' and 'solid persistent facial oedema of rosacea'. We report a patient with Morbihan disease showing persistent lymphoedema on the upper half of the face accompanied by unique histological findings of striking dermal dilated lymphatics and damage of the lymphatics at the site of the adjacent epithelioid cell granulomas, with histiocytes bulging into the lymphatic lumen. The marked epithelioid cell granulomas forming around dermal lymphatic vessels with subsequent lymphatic damage and luminal obstruction by histiocytic infiltration may account for the development of lymphoedema in this patient.
DOI: 10.1111/j.1365-2230.2008.02892.x
PubMed: 18627384
Affiliations:
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pubmed:18627384Le document en format XML
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<term>Histiocytes (immunology)</term>
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<term>Lymphedema (immunology)</term>
<term>Male</term>
<term>Rosacea (immunology)</term>
<term>Skin (immunology)</term>
<term>Syndrome</term>
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<term>Dermatoses faciales (immunologie)</term>
<term>Granulome (immunologie)</term>
<term>Histiocytes (immunologie)</term>
<term>Humains</term>
<term>Lymphoedème (immunologie)</term>
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<term>Sujet âgé</term>
<term>Syndrome</term>
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<term>Dermatoses faciales</term>
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<term>Lymphoedème</term>
<term>Peau</term>
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<term>Vaisseaux lymphatiques</term>
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<term>Sujet âgé</term>
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<front><div type="abstract" xml:lang="en">Morbihan disease is a rare complication of rosacea, characterized by persistent lymphoedema on the upper half of the face, occurring during the chronic clinical course of rosacea. This refractory condition has been also designated as 'rosacea lymphoedema' and 'solid persistent facial oedema of rosacea'. We report a patient with Morbihan disease showing persistent lymphoedema on the upper half of the face accompanied by unique histological findings of striking dermal dilated lymphatics and damage of the lymphatics at the site of the adjacent epithelioid cell granulomas, with histiocytes bulging into the lymphatic lumen. The marked epithelioid cell granulomas forming around dermal lymphatic vessels with subsequent lymphatic damage and luminal obstruction by histiocytic infiltration may account for the development of lymphoedema in this patient.</div>
</front>
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