Heer-Sonderhoff A H, Arning M, Wehmeier A, Südhoff T, Zumdick M
Clinic for Hematology, Oncology and Clinical Immunology, Heinrich-Heine-University, Düsseldorf, Germany.
Ann Hematol. 1995 Nov;71(5):257-61. doi: 10.1007/BF01744376.
Acute febrile neutrophilic dermatosis (AFND, Sweet's syndrome) is clinically characterized by fever, neutrophilic leukocytosis, and tender dermal plaques. Histological examination typically reveals infiltration of the dermis by neutrophils. In three patients (2 female, 1 male, 54-59 years) with acute leukemia (2 myelogenous, 1 lymphoblastic) dermal plaques developed during febrile episodes in chemotherapy-induced pancytopenia. The clinical appearance was compatible with AFND. The diagnosis was substantiated by skin biopsies which showed dense neutrophilic dermal infiltrates without leukemic cells. Leukocytoclastic vasculitis was considered as differential diagnosis. Plasma levels of soluble adhesion molecules ICAM-1, VCAM-1, and E-selectin regulating leukocyte transendothelial migration were in the normal range. Systemic glucocorticoids were avoided because of the high risk of infection during prolonged bone marrow aplasia. The lesions were treated with topical steroids and resolved without scarring within 1-5 weeks. AFND has been reported in association with acute leukemia at normal or elevated white blood cell counts. Although implausible from a pathophysiological point of view, similar neutrophilic dermal infiltrates were found in three patients during chemotherapy-induced pancytopenia with white blood cell counts distinctly below 1 x 10(9)/l.
急性发热性嗜中性皮病(AFND,Sweet综合征)的临床特征为发热、嗜中性白细胞增多及压痛性皮肤斑块。组织学检查通常显示真皮有嗜中性粒细胞浸润。3例急性白血病患者(2例女性,1例男性,年龄54 - 59岁,2例为髓性白血病,1例为淋巴细胞白血病)在化疗引起全血细胞减少的发热期出现皮肤斑块。临床表现符合AFND。皮肤活检证实了诊断,活检显示真皮有密集的嗜中性粒细胞浸润但无白血病细胞。白细胞破碎性血管炎被视为鉴别诊断。调节白细胞跨内皮迁移的可溶性黏附分子ICAM - 1、VCAM - 1和E - 选择素的血浆水平在正常范围内。由于长期骨髓再生障碍期间感染风险高,避免使用全身糖皮质激素。皮损用外用类固醇治疗,1 - 5周内消退且无瘢痕形成。AFND已被报道与白细胞计数正常或升高的急性白血病相关。尽管从病理生理学角度来看不太可能,但在3例化疗引起全血细胞减少且白细胞计数明显低于1×10⁹/L的患者中发现了类似的嗜中性粒细胞真皮浸润。