Syn.: Lymphomatoid granulomatosis.
Def .: Lymphoma with perivascular infiltrates and symptoms of severe tissue damage.
Epid .: It occurs in adult males.
Etiol .: Not known, related to EBV infection.
Localization: The area of the nose and palate, it develops from an extra-nodal location, it affects the kidneys, the CNS and the lungs.
Clinical: Development of a mid-face lesion with skin involvement and red-brown nodules, with signs of rapid disintegration; is accompanied by lung involvement.
Hist .: Cells from the lymphocyte lineage around the vessel wall, presence of large atypical cells, eosinophils, plasmocytes, extensive necrosis, fibrin deposits and signs of fibrosis.
DL: Leather clippings.
DI. T cells expressing CD4 + or CD8 + typically CD3-, CD56 +.
DR: Wegener’s granulomatosis.
Heal: Chemotherapy.
Year: Very bad, death within 2-3 years.
Lit.: [1] Cheung MMC, Chan JKC, Lau WH i wsp.: Primary non-Hodgkin lymphoma of the nose and nosopharynx: clinical features tumor immunophenotype and treatment outcome in 113 patients. J Clin Oncol 16, 70-77, 1998. [2] El Shabrawi-Caelen L, Cerrono L, Kerl H: The clinicopathologic spectrum of cytotoxic lymphomas of the skin. Skin Cut Med Surg 2000, 10; 118-23
Source: A. Kaszuba, Z. Adamski: “Lexicon of dermatology”; XNUMXst edition, Czelej Publishing House