Chaudhary Savita, Bansal Cherry, Ranga Upasna, Singh Kk
Department of Dermatology, Era's Lucknow Medical College and Hospital, Lucknow 226003, Uttar Pradesh, India.
Ecancermedicalscience. 2013 Aug 5;7:337. doi: 10.3332/ecancer.2013.337. Print 2013.
Mycosis fungoides (MF) is the most common variant of primary cutaneous T-cell lymphoma (CTCL). It is generally associated with an indolent clinical course and characterised by well-defined clinicopathological features. Although rare, CTCLs constitute 65% of all cutaneous lymphoid malignancies, of which 50% are patients with MF. The erythrodermic variants of MF, a malignancy of mature, skin homing and clonal T lymphocytes, usually present in mid to late adulthood. Association with hypereosinophilia is important in prognosis. We report a case of erythrodermic MF with hypereosinophilic syndrome in a 22-year-old female presenting with gradually progressive intractable erythroderma with intensely pruritic multiple papules, plaques, and nodules involving more than 90% of body surface area. Diagnosis was confirmed by histopathological examination and immunophenotyping from multiple skin biopsies.
蕈样肉芽肿(MF)是原发性皮肤T细胞淋巴瘤(CTCL)最常见的变异型。它通常与惰性临床病程相关,具有明确的临床病理特征。虽然罕见,但CTCL占所有皮肤淋巴恶性肿瘤的65%,其中50%是MF患者。MF的红皮病型变异型是成熟的、归巢于皮肤的克隆性T淋巴细胞的恶性肿瘤,通常出现在成年中期至晚期。与嗜酸性粒细胞增多症相关对预后很重要。我们报告一例22岁女性红皮病型MF合并嗜酸性粒细胞增多综合征,患者表现为逐渐进展的顽固性红皮病,伴有剧烈瘙痒的多个丘疹、斑块和结节,累及身体表面积超过90%。通过多次皮肤活检的组织病理学检查和免疫表型分析确诊。