Department of Dermatology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.
JAMA Dermatol. 2013 Jan;149(1):79-83. doi: 10.1001/2013.jamadermatol.548.
Neutrophilic dermatoses comprise a wide spectrum of inflammatory diseases with overlapping features characterized histologically by the presence of an aseptic neutrophilic infiltrate in the epidermis, dermis, and/or hypodermis and are often associated with systemic inflammatory and neoplastic disorders.
We describe 3 patients with an unusual neutrophilic dermatosis characterized by relapsing episodes of fever, widespread infiltrated plaques with bullous appearance, and variable involvement of the arms, legs, abdomen, and/or trunk. Light microscopy studies showed marked edema of the papillary dermis with an inflammatory infiltrate consisting mainly of mature neutrophils. All 3 patients were morbidly obese, and workup revealed underlying cancer in 2 cases: myeloma and breast carcinoma. Management of the underlying disease resulted in long-term remission of the skin disease.
The clinicopathologic features in our 3 cases best correspond to a widespread giant cellulitis-like form of Sweet syndrome. Knowledge of this newly observed unusual variant of Sweet syndrome within the broad spectrum of neutrophilic diseases is important for its prompt and proper management.
中性粒细胞皮肤病包括广泛的炎症性疾病谱，其特征为组织学上存在表皮、真皮和/或皮下组织的无菌性中性粒细胞浸润，常伴有全身炎症和肿瘤性疾病。
我们描述了 3 例具有不寻常中性粒细胞皮肤病的患者，其特征为反复发作的发热、广泛浸润性斑块伴水疱外观，以及手臂、腿部、腹部和/或躯干的不同程度受累。光镜研究显示，乳头真皮明显水肿，炎症浸润主要由成熟中性粒细胞组成。这 3 例患者均为病态肥胖，检查发现 2 例存在潜在癌症：骨髓瘤和乳腺癌。对基础疾病的治疗导致皮肤病的长期缓解。
我们 3 例患者的临床病理特征与广泛的巨细胞性蜂窝织炎样形式的Sweet 综合征最为相符。了解这种在广泛的中性粒细胞疾病谱中观察到的新的不寻常 Sweet 综合征变异，对于其及时和适当的管理非常重要。
