Primary cutaneous Epstein-Barr virus-related lymphoproliferative disorders in 4 immunosuppressed children.
Primary cutaneous Epstein-Barr virus-related lymphoproliferative disorders are rare. We describe 4 cases in children: two with acquired immunodeficiencies (HIV infection, heart transplantation) and two with congenital immunodeficiencies (ataxia-telangiectasia and an undetermined disease affecting the T lymphocytes). Two of the lymphoproliferative disorders were T-cell types and two were B-cell types. The two T-cell types were also Epstein-Barr virus positive, which is extremely rare. Three of the patients developed extracutaneous disease with poor outcome, resulting in death.
Wallet-Faber
Nadège
N
Department of Pathology, Groupe Hospitalier Necker Enfants-Malades, Assistance Publique-Hôpitaux de Paris, Université Paris-Descartes, Paris, France. nadegewallet@club-internet.fr
Bodemer
Christine
C
Blanche
Stéphane
S
Delabesse
Eric
E
Eschard
Catherine
C
Brousse
Nicole
N
Fraitag
Sylvie
S
D001402:B-Lymphocytes / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D017809:Fatal Outcome; D005260:Female; D015658:HIV Infections / Q000150:complications*; D016027:Heart Transplantation; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications*; D007165:Immunosuppression Therapy / Q000009:adverse effects*; D008232:Lymphoproliferative Disorders / Q000209:etiology* / Q000473:pathology / Q000821:virology; D008297:Male; D017193:Skin Diseases, Viral / Q000150:complications*; D013601:T-Lymphocytes / Q000473:pathology