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Lymphomatoid granulomatosis, a rare lymphoproliferative disorder, was previously defined by categorical pulmonary involvement with possible invasion into the skin, central nervous system, liver, and kidneys. However, recent reports have documented confirmed cases of lymphomatoid granulomatosis without lung involvement. Here, the authors describe a 70-year-old male with rheumatoid arthritis on methotrexate who presented with an ulcerating lesion on the right lower eyelid, initially suspicious for a basal cell carcinoma.

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An Update on EBV-related Cutaneous Lymphoproliferative Disorders: a Systematic Review.

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November 2024

Universidad de Zaragoza, Zaragoza, España; Servicio de Anatomía Patológica, Hospital Clínico Universitario Lozano Blesa, Zaragoza, España.

Epstein Barr virus (EBV) positive B lymphoproliferative disorders (LPD) with cutaneous involvement include a series of rare entities that go from indolent processes to aggressive lymphomas. B-cell EBV+ LPD mainly affect immunocompromised patients while T-cell EBV+ LPD are more prevalent in specific geographic regions such as Asia, Central America, and South America. Since the latest WHO-EORTC classification of cutaneous lymphomas in 2018, significant changes have been included in the new classifications of hematological malignancies.

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Article Synopsis
  • - Epstein Barr Virus (EBV) is linked to various cancers, including nasopharyngeal and gastric carcinomas, as well as different types of lymphomas that can range from mild to severe illnesses.
  • - The review highlights skin-related disorders associated with EBV, such as EBV-positive mucocutaneous ulcer, lymphomatoid granulomatosis, and several lymphomas including diffuse large B cell lymphoma and plasmablastic lymphoma.
  • - Due to the rarity of these skin conditions, a multidisciplinary approach and referrals to specialized care centers are recommended for proper management and treatment.
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Article Synopsis
  • * Histopathology revealed atypical lymphoid cells showing specific markers, raising concerns for anaplastic large cell lymphoma (ALCL), though the ulcer healed completely within three weeks.
  • * The authors conclude that this case represents a rare type of intraoral CD30+ T-cell LPD, which they believe corresponds to cutaneous LyP type C, highlighting its diagnostic challenges.
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