甜蜜综合征
坏疽性脓皮病
医学
鉴别诊断
病理
皮肤病科
炎症性肠病
疾病
免疫学
作者
Jérémie Delaleu,C. Lepelletier,A. Calugareanu,Adèle de Masson,E. Charvet,Arnaud Petit,Irina Giurgea,Serge Amselem,Sonia Karabina,M. Jachiet,Thibault Mahévas,Caroline Ram‐Wolff,Marie‐Dominique Vignon‐Pennamen,M. Bagot,Maxime Battistella,Jean‐David Bouaziz
标识
DOI:10.1016/j.revmed.2022.06.007
摘要
Neutrophilic dermatoses (ND) are a group of inflammatory skin conditions characterized by a neutrophilic infiltrate on histopathology with no evidence of infection. ND are classified based upon the localization of neutrophils within the skin and clinical features. Recent findings suggest that ND are due to two main mechanisms: i) a polyclonal hereditary activation of the innate immune system (polygenic or monogenic); or ii) a clonal somatic activation of myeloid cells such as encountered in myelodysplastic syndrome or VEXAS syndrome. ND belong to internal medicine as a great number of patients with ND suffer from an underlying condition (such as hematological malignancy, inflammatory bowel disease, auto-immune and auto-inflammatory diseases). ND are diagnoses of exclusion and physicians should always consider differential diagnoses, particularly skin infections. Here, we review the pathophysiology and classification of the main ND (i.e., subcorneal pustular dermatosis (Sneddon-Wilkinson Disease) and Intercellular IgA dermatoses, aseptic pustulosis of the folds, Sweet syndrome, neutrophilic eccrine hidradenitis, pyoderma gangrenosum, erythema elevatum diutinum, neutrophilic urticarial dermatosis and neutrophilic panniculitis), their clinical and histopathological features, and we highlight the investigations that are useful to identify ND-associated diseases and to exclude the differential diagnoses.
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