Lymphomatoid granulomatosis revealed by cutaneous lesions

• Published in: Annales de dermatologie et de vénéréologie Vol. 138; no. 8-9; pp. 591 - 596
• Main Authors: Quéreux, G, Brocard, A, Peuvrel, L, Knol, A-C, Renaut, J-J, Dréno, B
• Format: Journal Article
• Language: French
• Published: France 01.08.2011
• Subjects: Antineoplastic Combined Chemotherapy Protocols - administration & dosage; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; B-Lymphocytes - pathology; B-Lymphocytes - virology; Cough - etiology; Cyclophosphamide - administration & dosage; Doxorubicin - administration & dosage; Epstein-Barr Virus Infections - complications; Epstein-Barr Virus Infections - diagnosis; Fatal Outcome; Herpesvirus 4, Human - isolation & purification; Humans; Immunocompetence; Lung - pathology; Lung - virology; Lymphocytes, Tumor-Infiltrating - pathology; Lymphomatoid Granulomatosis - complications; Lymphomatoid Granulomatosis - diagnosis; Lymphomatoid Granulomatosis - drug therapy; Lymphomatoid Granulomatosis - virology; Male; Middle Aged; Prednisolone - administration & dosage; Skin - pathology; T-Lymphocytes - pathology; Vincristine - administration & dosage; Viral Load
• ISSN: 0151-9638
• DOI: 10.1016/j.annder.2011.02.017

Lymphomatoid granulomatosis is a rare Epstein Barr virus (EBV)-related lymphoproliferative disorder. It most frequently involves the lungs, skin and central nervous system and arises preferentially in patients with immune disorders. Here we report a case revealed by cutaneous lesions in an immunocompetent patient. A 56-year-old man consulted for erythematous nodules of the trunk associated with malaise and marked weight loss (14kg). In a few days the nodules became necrotic. Two weeks later a cough appeared and the chest computerized tomography showed multiple poorly defined nodular opacities with a peribronchovascular distribution. Cutaneous and pulmonary biopsies showed an infiltrate composed of medium-sized atypical lymphocytes T and B. EBV was present in the infiltrate (in situ hybridization) with a high EBV load in plasma. All of these data helped confirm the diagnosis of lymphoid granulomatosis. Despite aggressive treatment with polychemotherapy, the patient died after 2 months. Lymphomatoid granulomatosis represents a diagnostic challenge. In most cases, the presenting symptoms are not specific: malaise, weight loss, fever and cough. Moreover histology is difficult because of the T-cell-rich background. It is essential to consider this diagnosis in cases of cutaneous and pulmonary symptoms.