Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2022;11(2):89-102.
doi: 10.1007/s13671-022-00355-8. Epub 2022 Mar 16.

Neutrophilic Dermatoses: a Clinical Update

Emma H Weiss  1 , Christine J Ko  1 , Thomas H Leung  2 , Robert G Micheletti  2 , Arash Mostaghimi  3 , Sarika M Ramachandran  1 , Misha Rosenbach  2 , Caroline A Nelson  1   4
Affiliations
Review

Neutrophilic Dermatoses: a Clinical Update

Emma H Weiss et al. Curr Dermatol Rep. 2022.

Abstract

Purpose of review: Neutrophilic dermatoses are defined by the presence of a sterile neutrophilic infiltrate on histopathology. This review focuses on the pathogenesis, epidemiology, clinicopathological features, diagnosis, and management of four disorders: Sweet syndrome, pyoderma gangrenosum, Behçet syndrome, and neutrophilic eccrine hidradenitis.

Recent findings: Recent studies have provided insight into the complex pathogenesis of neutrophilic dermatoses. Evidence supports an intricate interplay of abnormal neutrophil function and inflammasome activation, malignant transformation into dermal infiltrating neutrophils, and genetic predisposition.

Summary: Neutrophilic dermatoses have diverse cutaneous and extracutaneous manifestations and may be associated with significant morbidity and mortality. Common underlying associations include infectious, inflammatory, and neoplastic disorders, as well as drug reactions. Emerging diagnostic and therapeutic frameworks identify an expanding role for biologic and targeted anti-inflammatory therapies.

Keywords: Acute febrile neutrophilic dermatosis; Behçet disease; Neutrophilic dermatosis; Neutrophilic eccrine hidradenitis; Pyoderma gangrenosum; Sweet syndrome.

PubMed Disclaimer

Conflict of interest statement

Conflict of InterestThe authors have no relevant financial or non-financial interests to disclose. Dr. Mostaghimi reports grants and personal fees from Pfizer, personal fees and other from Concert, personal fees and other from Lilly, personal fees from AbbVie, personal fees from ACON Labs, personal fees from Bioniz, personal fees from hims and hers, and personal fees from Digital Diagnostics, outside the submitted work. Dr. Rosenbach reports personal fees from Merck, personal fees from Janssen, personal fees from Novartis, personal fees from Eli Lilly, and grants and personal fees from Processa, outside the submitted work.

Figures

Fig. 1
Fig. 1
Sweet syndrome. A Edematous pink papules and plaques on the thigh. B Papillary dermal edema and an underlying dense infiltrate of mature neutrophils (40×, hematoxylin and eosin staining). C Higher magnification of mature neutrophils (400×, hematoxylin and eosin staining)
Fig. 2
Fig. 2
Pyoderma gangrenosum. A Irregular slate-gray ulcer with central bullae with an expanding violaceous to red border on the lower leg. The border may not be obviously violaceous in darker skin types. B Neutrophils predominate in the dermis below the ulceration (100×, hematoxylin and eosin staining). C The border of the ulcer shows a neutrophilic infiltrate in the dermis (400×, hematoxylin and eosin staining)
Fig. 3
Fig. 3
Behçet syndrome. A Oral aphthous ulcer. B Histopathologic findings can show an acute neutrophilic infiltrate, as with any ulcer (40×, hematoxylin and eosin staining)
Fig. 4
Fig. 4
Neutrophilic eccrine hidradenitis. A Erythematous macules and papules on the sole. B In this case associated with chemotherapy, there is vacuolar change and peri-eccrine inflammation (40×, hematoxylin and eosin staining). C Higher magnification shows a mixed infiltrate with scattered neutrophils and squamous metaplasia of the eccrine coils (400×, hematoxylin and eosin staining)
See this image and copyright information in PMC

References

    1. Bainton DF, Ullyot JL, Farquhar MG. The development of neutrophilic polymorphonuclear leukocytes in human bone marrow. J Exp Med. 1971;134(4):907–934. doi: 10.1084/jem.134.4.907. - DOI - PMC - PubMed
    1. Marzano AV, Ortega-Loayza AG, Heath M, Morse D, Genovese G, Cugno M. Mechanisms of inflammation in neutrophil-mediated skin diseases. Front Immunol. 2019;10:1059. doi: 10.3389/fimmu.2019.01059. - DOI - PMC - PubMed
    1. Nelson CA, Stephen S, Ashchyan HJ, James WD, Micheletti RG, Rosenbach M. Neutrophilic dermatoses. J Am Acad Dermatol. 2018;79(6):987–1006. doi: 10.1016/j.jaad.2017.11.064. - DOI - PubMed
    1. Heath MS, Ortega-Loayza AG. Insights into the pathogenesis of Sweet’s syndrome. Front Immunol. 2019;10:414. doi: 10.3389/fimmu.2019.00414. - DOI - PMC - PubMed
    1. Marzano AV, Cugno M, Trevisan V, Fanoni D, Venegoni L, Berti E, et al. Role of inflammatory cells, cytokines and matrix metalloproteinases in neutrophil-mediated skin diseases. Clin Exp Immunol. 2010;162(1):100–107. doi: 10.1111/j.1365-2249.2010.04201.x. - DOI - PMC - PubMed

LinkOut - more resources