Guillet S, Stokkermans J, Vergier B, Doutre M-S, Beylot-Barry M
Service de dermatologie, hôpital de Haut-Lévêque, CHU de Bordeaux, avenue de Magellan, 33600 Pessac, France.
Ann Dermatol Venereol. 2013 Oct;140(10):635-40. doi: 10.1016/j.annder.2013.04.088. Epub 2013 May 24.
Large-cell transformation in mycosis fungoides [MF] is associated with an aggressive clinical course and poor survival. In addition to tumour progression, infectious complications may be the cause of death. We report a case of aseptic pustular dermatosis in transformed MF that very quickly became aggressive and was followed by rapid death of the patient.
A 47-year-old man presented with disseminated CD30+ transformed MF skin tumours refractory to chemotherapy for nine consecutive months. Ten days after a first course of dexamethasone, cytarabine and carboplatin, he was hospitalized for febrile neutropenia. Following this episode of neutropenia, he developed a febrile pustular rash disseminated on the tumours and on plaques, associated with marked neutrophilic leucocytosis. Bacteriological and mycological skin and blood samples were negative. Tumour biopsies showed a dermal infiltrate of CD30+ T-cells corresponding to the transformed MF, mixed with neutrophils. The pustule biopsies revealed intra-epidermal pustules and dermal-epidermal infiltration of neutrophils. The pustular eruption regressed with corticosteroids. Despite a last line of treatment with romidepsin, the patient died 6 weeks after the "neutrophilic" event.
Episodes of neutrophilic dermatosis [ND] are frequently seen in haematological disease and some substances can induce a variant form of eccrine hidradenitis. However, our patient's presentation was different, without the appearance of "classic" ND but rather diffuse pustular lesions carrying a poor prognosis, which was followed by rapid death. Once the hypothesis of infectious complications has been ruled out, treatment is based on systemic corticosteroids associated with specific treatment of the lymphoma.
蕈样肉芽肿（MF）中的大细胞转化与侵袭性临床病程及不良生存相关。除肿瘤进展外，感染性并发症可能是死亡原因。我们报告1例转化型MF患者出现无菌性脓疱性皮肤病，病情迅速进展并导致患者快速死亡。
1例47岁男性，连续9个月接受化疗，其播散性CD30+转化型MF皮肤肿瘤疗效不佳。在接受第一疗程地塞米松、阿糖胞苷和卡铂治疗10天后，因发热性中性粒细胞减少症住院。中性粒细胞减少症发作后，他在肿瘤和斑块上出现发热性脓疱疹，并伴有明显的中性粒细胞增多。皮肤和血液样本的细菌学和真菌学检查均为阴性。肿瘤活检显示真皮内有CD30+T细胞浸润，符合转化型MF，伴有中性粒细胞。脓疱活检显示表皮内脓疱及真皮-表皮中性粒细胞浸润。脓疱疹经皮质类固醇治疗后消退。尽管使用罗米地辛进行了最后一线治疗，但患者在“中性粒细胞”事件发生6周后死亡。
中性粒细胞性皮肤病（ND）在血液系统疾病中常见，某些物质可诱发一种变异型小汗腺汗管炎。然而，我们患者的表现不同，未出现“经典”ND，而是出现预后不良的弥漫性脓疱病变，随后迅速死亡。一旦排除感染性并发症的假设，治疗基于全身性皮质类固醇联合淋巴瘤的特异性治疗。
