title: Lymphomatoid granulomatosis presenting with cutaneous involvement: a case report and review of the literature.
abstract: Lymphomatoid granulomatosis (LG) is a rare Epstein-Barr virus (EBV)-associated B-cell lymphoproliferative disorder presenting in middle adulthood that nearly always affects the lungs and shows cutaneous involvement in up to 50% of cases. Skin lesions are present at the time of diagnosis in roughly one-third of patients and may precede the development of lung lesions in as many as 10-15%. Recognition by both the dermatologist and dermatopathologist is therefore crucial for early and accurate diagnosis. While skin involvement is grossly and microscopically diverse, the disease most commonly presents as erythematous subcutaneous and dermal nodules showing the classic histopathologic triad of transmural lymphocytic angiitis, atypical B-lymphocytes in a polymorphous T-cell background, and necrotic foci within lymphoid aggregates. We present a case of lymphomatoid granulomatosis initially presenting with cutaneous lesions, with an accompanying review of the literature.
authors.affiliation: Department of Pathology, Carver College of Medicine, University of Iowa, Iowa City, IA, USA.
authors.forename: Carolyn D
authors.initials: CD
authors.lastname: Rysgaard
authors.affiliation: Department of Pathology, Carver College of Medicine, University of Iowa, Iowa City, IA, USA.|Department of Dermatology, Carver College of Medicine, University of Iowa, Iowa City, IA, USA.
authors.forename: Mary Seabury
authors.initials: MS
authors.lastname: Stone
mesh_terms: D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001402:B-Lymphocytes / Q000473:pathology; D001706:Biopsy; D003937:Diagnosis, Differential; D005260:Female; D004854:Herpesvirus 4, Human / Q000302:isolation & purification; D006801:Humans; D008175:Lung Neoplasms / Q000188:drug therapy / Q000473:pathology* / Q000821:virology; D008230:Lymphomatoid Granulomatosis / Q000188:drug therapy / Q000473:pathology* / Q000821:virology; D000069283:Rituximab / Q000627:therapeutic use; D012878:Skin Neoplasms / Q000188:drug therapy / Q000473:pathology* / Q000821:virology; D013601:T-Lymphocytes / Q000473:pathology; D014657:Vasculitis / Q000473:pathology
chemical_list: D000970:Antineoplastic Agents; D000069283:Rituximab
keywords: dermatopathology; granulomatous inflammation; lymphoma; vasculitis