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Neutrophilic dermatoses 

Neutrophilic dermatoses
Chapter:
Neutrophilic dermatoses
Source:
Oxford Textbook of Rheumatology (4 ed.)
Author(s):

Pia Moinzadeh

and Thomas Krieg

DOI:
10.1093/med/9780199642489.003.0166

Neutrophilic dermatoses (ND) comprise a group of non-infectious skin diseases characterized by a diffuse epidermal and/or dermal infiltrate consisting of polymorphonuclear neutrophilic inflammatory cells throughout the different skin layers. Depending on the localization of this infiltrate, patients may present with a variety of skin lesions such as pustules/vesiculopustules (epidermal infiltrate), plaques or papules (dermal infiltration), nodules or ulcerations (deep dermal/subcutaneous infiltrate) and also with possible systemic symptoms, such as leucocytosis, arthralgias, myalgia, and malaise. Depending on the localization of neutrophilic cells in the skin, the clinical picture of cutaneous lesions and further systemic symptoms the patients can be subdivided into different subgroups. These include Sweet’s syndrome (SS), pyoderma gangrenosum (PG), rheumatoid neutrophilic dermatitis, bowel-associated dermatosis-arthritis syndrome, subcorneal pustular dermatosis (Sneddon Wilkinson), acute generalized exanthematous pustulosis (AGEP), acrodermatitis continua of Hallopeau (ACH), palmoplantare pustuloses (PPP), pustular bacterid (PB), neutrophilic eccrine hidradenitis, and Behçet’s disease. The pathogenesis is still not fully elucidated but it has been hypothesized that it is associated with immunological dysfunctions, with abnormal cytokine signalling, causing an uncontrolled recruitment of neutrophilic cells. ND can be triggered by underlying systemic inflammatory diseases, malignancies, haematological disorders and/or medication use. Diagnostic procedures include detailed physical examination, laboratory tests, and histopathological assessments. The therapeutic management of ND includes systemic steroids in acute cases and immunosuppressive or immunomodulatory drugs in chronic forms. Underlying systemic conditions have to be diagnosed and treated.

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