Department of Medicine, Johns Hopkins University School of Medicine, 600 N. Wolfe Street, Baltimore, MD, 21287, USA.
Department of Radiology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
J Gen Intern Med. 2019 Nov;34(11):2669-2674. doi: 10.1007/s11606-019-05231-z. Epub 2019 Aug 6.
Erythroderma refers to a spectrum of skin diseases resulting in diffuse erythema and scaling encompassing ≥ 90% of the body surface area. The differential diagnosis ranges from primary dermatologic diseases such as atopic dermatitis and psoriasis to potentially deadly causes such as staphylococcal toxic shock syndrome, toxic epidermal necrolysis, and malignancy. Cutaneous T cell lymphoma (CTCL) is an uncommon but highly morbid cause of erythroderma. This non-Hodgkin lymphoma remains a diagnostic challenge due to its variable clinical presentation and varied histologic features. Mycosis fungoides (MF) is the most common form of CTCL. Making a timely diagnosis is challenging as it may mimic inflammatory diseases of the skin including eczema, psoriasis, lichen planus, and cutaneous lupus. We present a case of a 58-year-old man who presented with 5 years of cutaneous symptoms and several months of fevers and night sweats, ultimately diagnosed as MF. Owing to diffuse CD30 positivity, he was a candidate for brentuximab vedotin, an antibody-drug conjugate medication that selectively targets the CD30 antigen. This resulted in an excellent therapeutic response.
红皮病是指一系列导致皮肤弥漫性红斑和鳞屑的疾病，累及面积≥90%。鉴别诊断范围从特应性皮炎和银屑病等原发性皮肤病到葡萄球菌中毒性休克综合征、中毒性表皮坏死松解症和恶性肿瘤等潜在致命原因。皮肤 T 细胞淋巴瘤（CTCL）是一种罕见但高度致命的红皮病原因。由于其可变的临床表现和不同的组织学特征，这种非霍奇金淋巴瘤仍然是一个诊断挑战。蕈样肉芽肿（MF）是 CTCL 最常见的形式。及时做出诊断具有挑战性，因为它可能模仿包括湿疹、银屑病、扁平苔藓和皮肤狼疮在内的皮肤炎症性疾病。我们报告了一例 58 岁男性，他有 5 年的皮肤症状和几个月的发热和盗汗，最终被诊断为 MF。由于弥漫性 CD30 阳性，他是 Brentuximab vedotin 的候选者，Brentuximab vedotin 是一种抗体药物偶联药物，可选择性靶向 CD30 抗原。这导致了极好的治疗反应。
