Urticarial mycosis fungoides: A distinctive presentation with blood involvement and a peculiar immunophenotype

IF 1.6 4区 医学 Q3 DERMATOLOGY
Juan Torre-Castro, Concepción Postigo, Salma Machan, Margarita Estela Jo-Velasco, Javier Díaz de la Pinta, Jose Luis Rodríguez-Peralto, Raúl Córdoba, Luis Requena, Socorro María Rodríguez-Pinilla
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引用次数: 0

Abstract

Mycosis fungoides (MF) has been widely reported to mimick a considerable number of different dermatoses, including scarring alopecia, bullous dermatoses or cysts, and comedones. In atypical presentations, histopathology is essential for the diagnosis. We present two cases of MF with clinical urticarial lesions and a striking blood involvement that responded to mogamulizumab treatment. Histopathologically, both cases had classic MF features and shared a peculiar immunophenotype, with positivity for CD25 and FOXP3. Differential diagnoses included urticarial lymphomatoid drug reactions and other lymphomas, like T-cell prolymphocytic leukemia, atypical Sézary syndrome, or adult T-cell lymphocytic leukemia. A low suspicion threshold is necessary for the diagnosis of atypical presentations of MF.

荨麻疹性真菌病:具有血液受累和特殊免疫表型的独特表现。
据广泛报道,真菌病(MF)可模拟多种不同的皮肤病,包括瘢痕性脱发、牛皮癣或囊肿以及粉刺。对于非典型表现,组织病理学诊断至关重要。我们介绍了两例临床上伴有荨麻疹皮损和明显血液受累的多发性骨髓瘤病例,这两例病例对莫甘舒单抗治疗均有反应。从组织病理学上看,两例病例均具有典型的 MF 特征,并具有特殊的免疫表型,CD25 和 FOXP3 阳性。鉴别诊断包括荨麻疹性淋巴瘤样药物反应和其他淋巴瘤,如T细胞原淋巴细胞白血病、非典型塞扎里综合征或成人T细胞淋巴细胞白血病。诊断多发性骨髓瘤的非典型表现需要较低的怀疑门槛。
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来源期刊
CiteScore
3.20
自引率
5.90%
发文量
174
审稿时长
3-8 weeks
期刊介绍: Journal of Cutaneous Pathology publishes manuscripts broadly relevant to diseases of the skin and mucosae, with the aims of advancing scientific knowledge regarding dermatopathology and enhancing the communication between clinical practitioners and research scientists. Original scientific manuscripts on diagnostic and experimental cutaneous pathology are especially desirable. Timely, pertinent review articles also will be given high priority. Manuscripts based on light, fluorescence, and electron microscopy, histochemistry, immunology, molecular biology, and genetics, as well as allied sciences, are all welcome, provided their principal focus is on cutaneous pathology. Publication time will be kept as short as possible, ensuring that articles will be quickly available to all interested in this speciality.
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