Torri O, Ruto F, Dierick A, Labeille B, Lok C, Desablens B, Denoeux J P
Service de Dermato-Vénéréologie, CHU, Amiens.
Ann Dermatol Venereol. 1993;120(12):884-8.
the association of acute febrile neutrophilic dermatosis (Sweet's syndrome) with malignant haemopathies is well known and characterized by an usual lack of hyperleukocytosis: indeed, moderate neutropenia is often reported. However, cases of Sweet's syndrome in the agranulocytosis stage are exceptional (7 in the literature).
CASE-REPORT: We report the case of a woman with acute myeloblastic leukaemia who had presented with Sweet's syndrome in the phase of therapeutic aplasia during induction of treatment, in the absence of white blood cells transfusion or treatment with haematopoietic growth factor (GM CSF, GCSF).
the physiopathology of Sweet's syndrome is unknown. Various mechanisms have been suggested, including immune reaction type III, increased interleukin-1 synthesis, increased chemotaxis of neutrophils, action of haematopoietic growth factors, iatrogenic effect of some drugs (e.g. cotrimoxazole, furosemide or minocycline). Yet none of these mechanisms involving circulating polymorphonuclears or their bone marrow precursors can explain the occurrence of Sweet's syndrome in the phase of agranulocytosis.
the diagnosis of Sweet's syndrome must be considered in patients with agranulocytosis in order to avoid ineffective antibiotics and to initiate a corticosteroid therapy that will accelerate the cure of this benign dermatosis.
急性发热性嗜中性皮病(Sweet综合征)与恶性血液病的关联已为人熟知,其特点通常是缺乏白细胞增多症:实际上,常报告有中度中性粒细胞减少。然而,粒细胞缺乏症阶段的Sweet综合征病例极为罕见(文献中仅有7例)。
我们报告一例急性髓细胞性白血病女性患者,在诱导治疗期间的治疗性再生障碍期出现Sweet综合征,在此期间未进行白细胞输血或使用造血生长因子(粒细胞巨噬细胞集落刺激因子、粒细胞集落刺激因子)治疗。
Sweet综合征的病理生理学尚不清楚。已提出多种机制,包括III型免疫反应、白细胞介素 -1合成增加、中性粒细胞趋化性增加、造血生长因子的作用、某些药物(如复方新诺明、呋塞米或米诺环素)的医源性效应。然而,这些涉及循环多形核细胞或其骨髓前体的机制均无法解释粒细胞缺乏症阶段Sweet综合征的发生。
粒细胞缺乏症患者必须考虑Sweet综合征的诊断,以避免使用无效的抗生素,并启动皮质类固醇治疗,这将加速治愈这种良性皮肤病。