Rysgaard Carolyn D, Stone Mary Seabury
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.
J Cutan Pathol. 2015 Mar;42(3):188-193. doi: 10.1111/cup.12402. Epub 2014 Dec 8.
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.
淋巴瘤样肉芽肿病(LG)是一种罕见的与EB病毒(EBV)相关的B细胞淋巴增殖性疾病,好发于中年,几乎总是累及肺部,高达50%的病例有皮肤受累。约三分之一的患者在诊断时即有皮肤病变,多达10%-15%的患者皮肤病变可能先于肺部病变出现。因此,皮肤科医生和皮肤病理学家的识别对于早期准确诊断至关重要。虽然皮肤受累在大体和显微镜下表现多样,但该病最常见的表现为红斑性皮下和真皮结节,显示出经典的组织病理学三联征:透壁淋巴细胞性血管炎、多形性T细胞背景中的非典型B淋巴细胞以及淋巴样聚集区内的坏死灶。我们报告一例最初表现为皮肤病变的淋巴瘤样肉芽肿病病例,并对相关文献进行回顾。