Dogra Sunil, Mahajan Rahul
Department of Dermatology, Venereology and Leprology, Post-Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.
Indian J Dermatol Venereol Leprol. 2015 Mar-Apr;81(2):124-35. doi: 10.4103/0378-6323.152169.
Both phototherapy and photochemotherapy have been used in all stages of mycosis fungoides since they improve the symptoms and have a favourable adverse effect profile.
We performed an extensive search of published literature using keywords like "phototherapy", "photochemotherapy", "NBUVB", "PUVA", "UVA1", "mycosis fungoides", and "Sezary syndrome", and included systematic reviews, meta-analysis, national guidelines, randomized controlled trials (RCTs), prospective open label studies, and retrospective case series. These were then arranged according to their levels of evidence.
Five hundred and forty three studies were evaluated, of which 107 fulfilled the criteria for inclusion in the guidelines.
Photochemotherapy in the form of psoralens with ultraviolet A (PUVA) is a safe, effective, and well tolerated first line therapy for the management of early stage mycosis fungoides (MF), that is, stage IA, IB, and IIA (Level of evidence 1+, Grade of recommendation B). The evidence for phototherapy in the form of narrow-band UVB (NB-UVB) is less robust (Level of evidence 2++, Grade of recommendation B) but may be considered at least as effective as PUVA in the treatment of early-stage MF as an initial therapy. In patients with patches and thin plaques, NB-UVB should be preferentially used. PUVA may be reserved for patients with thick plaques and those who relapse after initial NB-UVB therapy. For inducing remission, three treatment sessions per week of PUVA phototherapy or three sessions per week of NB-UVB phototherapy may be advised till the patient achieves complete remission. In cases of relapse, patients may be started again on PUVA monotherapy or PUVA may be combined with adjuvants like methotrexate and interferon (Level of evidence 2+, Grade of recommendation B). Patients with early-stage MF show good response to combination treatments like PUVA with methotrexate, bexarotene or interferon-α-2b. However, whether these combinations hold a significant advantage over monotherapy is inconclusive. For late stage MF, the above-mentioned combination therapy may be used as first-line treatment (Level of evidence 3, Grade of recommendation C). Currently, there is no consensus regarding maintenance therapy with phototherapy once remission is achieved. Maintenance therapy should not be employed for PUVA routinely and may be reserved for patients who experience an early relapse after an initial course of phototherapy (Level of evidence 2+, Grade of recommendation B). Bath-water PUVA may be tried as an alternative to oral PUVA in case the latter cannot be administered as the former may show similar efficacy (Level of evidence 2-, Grade of recommendation C). In pediatric MF and in hypopigmented MF, both NB-UVB and PUVA may be tried (Level of evidence 3, Grade of recommendation D).
光疗和光化学疗法已应用于蕈样肉芽肿的各个阶段，因为它们能改善症状且不良反应较少。
我们使用“光疗”“光化学疗法”“窄谱中波紫外线（NBUVB）”“补骨脂素联合紫外线A（PUVA）”“长波紫外线1（UVA1）”“蕈样肉芽肿”和“塞扎里综合征”等关键词对已发表的文献进行了广泛检索，纳入了系统评价、荟萃分析、国家指南、随机对照试验（RCT）、前瞻性开放标签研究和回顾性病例系列。然后根据证据水平对这些研究进行了整理。
共评估了543项研究，其中107项符合纳入指南的标准。
补骨脂素联合紫外线A（PUVA）形式的光化学疗法是治疗早期蕈样肉芽肿（MF），即IA期、IB期和IIA期的一种安全、有效且耐受性良好的一线治疗方法（证据水平1 +，推荐等级B）。窄谱中波紫外线（NB - UVB）形式的光疗证据不太充分（证据水平2 ++，推荐等级B），但可被视为与PUVA在治疗早期MF作为初始治疗时至少同样有效。对于有斑片和薄斑块的患者，应优先使用NB - UVB。PUVA可用于有厚斑块的患者以及初始NB - UVB治疗后复发的患者。为诱导缓解，建议每周进行3次PUVA光疗或每周进行3次NB - UVB光疗，直至患者完全缓解。在复发的情况下，患者可再次开始PUVA单一疗法，或者PUVA可与甲氨蝶呤和干扰素等辅助药物联合使用（证据水平2 +，推荐等级B）。早期MF患者对PUVA与甲氨蝶呤、贝沙罗汀或干扰素 - α - 2b等联合治疗反应良好。然而，这些联合治疗是否比单一疗法具有显著优势尚无定论。对于晚期MF，上述联合治疗可作为一线治疗方法（证据水平3，推荐等级C）。目前，关于缓解后光疗维持治疗尚无共识。PUVA通常不应常规用于维持治疗，可保留给在初始光疗疗程后早期复发的患者（证据水平2 +，推荐等级B）。如果无法口服PUVA，可尝试浴水PUVA作为替代方法，因为前者可能显示出相似的疗效（证据水平2 -，推荐等级C）。对于儿童MF和色素减退性MF，NB - UVB和PUVA均可尝试（证据水平3，推荐等级D）。
