Department of Dermatology, Boston University, Boston, MA, United States of America.
Department of Pathology and Laboratory Medicine, Lifespan-Rhode Island Hospital, Providence, RI, United States of America.
PLoS One. 2020 Feb 12;15(2):e0228751. doi: 10.1371/journal.pone.0228751. eCollection 2020.
Primary cutaneous CD30+ lymphoproliferative disorders (CD30CLPD) are the second most common type of cutaneous T cell lymphoma (CTCL) and include lymphomatoid papulosis (LyP) and primary cutaneous anaplastic large cell lymphoma (pcALCL). Case reports and small patient series suggest an association of CD30CLPD with atopic disorders. However, the prevalence of atopy in patients with CD30CLPD in retrospective studies depends on patients' recall which is not always reliable. More objective criteria of atopy include evidence of skin reactivity to allergens (positive prick test) and evidence of allergen-specific IgE in serum. This study was undertaken to test the hypothesis that atopy is prevalent in patients with CD30CLPD using serologic criteria of allergen-specific IgE antibodies to aeroallergens and Staphylococcal aureus enterotoxin superantigens (SSAgs).
We tested serum samples of CD30CLPD for common IgE-specific airborne allergens with the Phadiatop test, which if positive, is regarded as serologic evidence of atopy in adults. Sera were also tested for IgE antibodies reactive to three Staphylococcal enterotoxins with superantigenic properties (SSAg-IgE). Control sera were obtained from adult subjects evaluated for rhino-sinusitis and a negative Phadiatop test. Patients' history of an atopic disorder was obtained by retrospective chart review.
Nearly 50% of patients with the most common LyP types (A and C) had a positive Phadiatop test for allergic sensitization to common airborne allergens, and total serum IgE (IgE-t) was increased compared to non-atopic controls. At the IgE antibody concentration generally used to define serologic atopy (≥ 0.35 kUA/L), 8/31 (26%) samples of CD30CLPD and 7/28 (25%) samples of LyP were reactive to at least one SSAg-IgE compared to 3/52 (6%) control specimens (P = 0.016 and P = 0.028, respectively). TSST1-IgE was detected in 7 (23%) specimens of CD30CLPD, often together with SEB-IgE; SEA-IgE ≥ 0.35 kUA/L was not detected. For control specimens, TSST1-IgE exceeded the 0.35 kUA/L threshold in 3 (6%) specimens.
Patients with LyP types A and C have serologic evidence of atopy against common airborne antigens and SSAgs when compared to control adult subjects who had rhino-sinusitis and a negative Phadiatop test for aero-IgEs. Serologic evidence of atopy exceeded that determined by LyP patients' personal history. The findings support our hypothesis that an atopic diathesis may contribute to the pathogenesis of the most common types of LyP (A and C).
原发性皮肤 CD30+淋巴增生性疾病（CD30CLPD）是第二常见的皮肤 T 细胞淋巴瘤（CTCL），包括蕈样肉芽肿（LyP）和原发性皮肤间变性大细胞淋巴瘤（pcALCL）。病例报告和小系列患者研究表明 CD30CLPD 与特应性疾病有关。然而，在回顾性研究中，CD30CLPD 患者的特应性患病率取决于患者的回忆，而回忆并不总是可靠的。特应性的更客观标准包括对过敏原的皮肤反应证据（阳性划痕试验）和血清中过敏原特异性 IgE 的证据。本研究旨在通过对空气过敏原的过敏原特异性 IgE 抗体的血清学标准来检验特应性在 CD30CLPD 患者中普遍存在的假设，使用的标准为对空气传播过敏原和金黄色葡萄球菌肠毒素超抗原（SSAgs）的 Phadiatop 试验。
我们用 Phadiatop 试验检测 CD30CLPD 血清样本中常见的 IgE 特异性空气传播过敏原，如果阳性，则被认为是成人特应性的血清学证据。还检测了血清中对三种具有超抗原特性的金黄色葡萄球菌肠毒素（SSAg-IgE）的 IgE 抗体反应。对照血清取自接受鼻-鼻窦炎评估且 Phadiatop 试验阴性的成年受试者。通过回顾性病历审查获得患者特应性疾病史。
近 50%的最常见 LyP 类型（A 和 C）患者对常见空气传播过敏原的过敏致敏呈 Phadiatop 试验阳性，与非特应性对照相比，总血清 IgE（IgE-t）升高。在通常用于定义血清特应性的 IgE 抗体浓度（≥0.35 kUA/L）下，31 份 CD30CLPD 样本中有 8/31（26%）和 28 份 LyP 样本中有 8/28（25%）对至少一种 SSAg-IgE 呈反应，而 52 份对照样本中有 3/52（6%）（P=0.016 和 P=0.028）。在 7（23%）份 CD30CLPD 样本中检测到 TSST1-IgE，通常与 SEB-IgE 一起；SEA-IgE≥0.35 kUA/L 未检出。对于对照标本，3（6%）份标本中的 TSST1-IgE 超过 0.35 kUA/L 阈值。
与患有鼻-鼻窦炎且 Phadiatop 试验对空气 IgE 阴性的成年对照相比，A 型和 C 型 LyP 患者对常见空气抗原和 SSAgs 具有血清学特应性证据。特应性的血清学证据超过了 LyP 患者个人病史所确定的证据。这些发现支持我们的假设，即特应性倾向可能有助于最常见类型的 LyP（A 和 C）的发病机制。
