A Severe Case of Lymphomatoid Papulosis Type E Successfully Treated with Interferon-Alfa 2a.
A Severe Case of Lymphomatoid Papulosis Type E Successfully Treated with Interferon-Alfa 2a.
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DOI：
10.1155/2017/3194738
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发表时间：
2017
影响因子：
--
通讯作者：
Uzun S
中科院分区：
文献类型：
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作者：
Bilgiç Temel A;Unal B;Erdi Şanlı H;Duygulu Ş;Uzun S
淋巴瘤样丘疹病（LyP）是一种良性丘疹结节性皮疹，具有恶性淋巴瘤的组织学特征。一种新的LyP变异体被称为“E型”，最近被描述为与血管中心性和血管破坏性T细胞淋巴瘤具有相似的临床和组织学特征。LyP E型的特征是复发性丘疹结节性病变，其迅速转变为出血性坏死性溃疡，并通过留下疤痕而自发消退。现有的治疗方法都不影响LyP的自然病程。对于治疗，已经使用了各种方式，例如局部和全身类固醇、PUVA、甲氨蝶呤、贝沙罗汀和IFN α-2b。在这里，我们提出了一个严重的和毁灭性的情况下，一个非常罕见的变异的LyP E型，这是，据我们所知，第一例成功治疗IFN α-2a。现在疾病已经维持了六个月的缓解状态。
Lymphomatoid papulosis (LyP) is a benign papulonodular skin eruption with histologic features of malignant lymphoma. A new variant of LyP which was termed “type E” was recently described with similar clinical and histological features to angiocentric and angiodestructive T-cell lymphoma. LyP type E is characterized with recurrent papulonodular lesions which rapidly turn into hemorrhagic necrotic ulcers and spontaneous regression by leaving a scar. None of the available treatment modalities affects the natural course of LyP. For therapy various modalities have been used such as topical and systemic steroids, PUVA, methotrexate, bexarotene, and IFN alfa-2b. Here we present a severe and devastating case with a very rare variant of LyP type E, which is, to our knowledge, the first case successfully treated with IFN alfa-2a. Now disease has been maintaining its remission status for six months.