Perniciaro C, Zalla M J, White J W, Menke D M
Department of Dermatology, Mayo Clinic, Jacksonville, Fla.
Arch Dermatol. 1993 Sep;129(9):1171-6. doi: 10.1001/archderm.129.9.1171.
Peripheral T-cell lymphomas involving subcutaneous tissue are rare. With modern immunohistochemical stains, subcutaneous T-cell lymphomas may be demonstrated in patients previously thought to have fatal Weber-Christian disease, malignant histiocytosis, and histiocytic cytophagic panniculitis.
We describe two young women with subcutaneous lymphomas. They presented with erythematous nodular lesions, which suggested benign panniculitis. Skin biopsy specimens in both patients demonstrated large atypical lymphocytes in the subcutaneous tissue, karyorrhexis, and cytophagia. Immunohistochemical studies confirmed a T-cell phenotype. Molecular genetics studies performed in one patient showed a major T-cell clonal population.
Subcutaneous T-cell lymphoma is a rare, distinct subtype of postthymic peripheral T-cell lymphoma. Cytophagic "histiocytic" panniculitis is frequently a prominent histologic feature. Early systemic chemotherapy may offer therapeutic advantages in patients with this disease.
累及皮下组织的外周T细胞淋巴瘤较为罕见。借助现代免疫组化染色，皮下T细胞淋巴瘤可见于既往被认为患有致命性韦格纳-克里斯蒂安病、恶性组织细胞增多症及组织细胞吞噬性脂膜炎的患者。
我们描述了两名患有皮下淋巴瘤的年轻女性。她们表现为红斑性结节性病变，提示为良性脂膜炎。两名患者的皮肤活检标本均显示皮下组织中有大量非典型淋巴细胞、核碎裂及细胞吞噬现象。免疫组化研究证实为T细胞表型。对其中一名患者进行的分子遗传学研究显示存在主要的T细胞克隆群体。
皮下T细胞淋巴瘤是胸腺后外周T细胞淋巴瘤中一种罕见的独特亚型。吞噬性“组织细胞性”脂膜炎常常是突出的组织学特征。早期全身化疗可能对患有此病的患者具有治疗优势。
