Open AccessLeg‐type form of idiopathic multicentric Castleman disease associated with severe lower extremity chronic venous/lymphatic disease
Author(s) -
Ballul Thomas,
Belfeki Nabil,
Masson Adèle,
Meignin Véronique,
Woerther PaulLouis,
Martin Antoine,
Poullot Elsa,
Wargnier Alain,
Fadlallah Jehane,
Garzaro Margaux,
Malphettes Marion,
Fieschi Claire,
Maisonobe Lucas,
Bensekhri Hayat,
Guillot Hélène,
Bertinchamp Rémi,
Jachiet Marie,
Poirot Justine,
Galicier Lionel,
Oksenhendler Eric,
Boutboul David
Publication year - 2022
Publication title -
ejhaem
Language(s) - English
Resource type - Journals
ISSN - 2688-6146
DOI - 10.1002/jha2.353
Subject(s) - medicine , disease , etiology , rituximab , lymphatic system , pathological , pathogenesis , lymph , pathology , immunology , lymphoma
Idiopathic multicentric Castleman disease (iMCD) is a lymphoproliferative disease of unknown etiology. Deciphering mechanisms involved in CD pathogenesis may help improving patients’ care. Six cases of stereotyped sub‐diaphragmatic iMCD affecting lower limb‐draining areas and associated with severe and often ulcerative lower extremity chronic dermatological condition were identified in our cohort. Pathological examination revealed mixed or plasma‐cell type MCD. In three patients, shotgun metagenomics failed to identify any pathogen in involved lymph nodes. Antibiotics had a suspensive effect while rituximab and tocilizumab failed to improve the condition. This novel entity requires a specific approach and exclusion of potentially harmful immunomodulation.