Neutrophilic dermatoses

Summarizing term for an etiopathogenetically and clinically heterogeneous group of diseases characterized by varying degrees of systemic symptoms that are often febrile, reactive and frequently arthritic. The associated skin changes are defined histopathologically by neutrophilic diffuse dermatitis without evidence of vasculitis (exclusion criterion). On the one hand,"acute febrile neutrophilic dermatosis (Sweet syndrome)" is to be regarded as the prototype of this group of diseases. On the other hand, autoinflammatory (non-bacterial) pustuloses belong to this group of diseases.

In the following, "neutrophilic" dermatoses are listed in alphabetical order, whereby the classification into "in the broader and narrower sense" must be considered provisional. "Neutrophilic" dermatoses are often accompanied by reactive arthritis (see also dermatitis-arthritis syndromes) or they are characterized by a primarily arthritic constellation. They have also been described as ADRs during treatment with TNF-alpha inhibitors (Calabrese L et al. 2024)

Neutrophilic dermatoses (in the narrower sense):

• Acute neutrophilic dermatosis (Sweet syndrome)
• Chronic recurrent annular neutrophilic dermatosis (CRAND)
• Dermatitis, rheumatoid, neutrophilic
• Neutrophilic figured erythema in childhood
• Dermatitis, interstitial, granulomatous with arthritis
• Dermatosis, acute febrile neutrophilic (Sweet syndrome)
• Hidradenitis, neutrophilic, eccrine
• Erythema anulare rheumaticum
• Impetigo herpetiformis
• Psoriasis pustulosa generalisata
• Pustulosis, subcorneal (subcorneal pustular dermatosis)
• Neutrophilic dermatosis of the back of the hand
• Pyoderma gangraenosum
• Melanosis neonatal transient pustular
• Wegener's granulomatosis
• See also under Autoinflammatory syndromes (monogenic) skin changes

Neutrophilic dermatoses (in the broader sense):

• Acne fulminans
• Arthroosteitis, pustular
• Acropustulosis, infantile
• Anticonvulsant hypersensitivity syndrome
• Behçet, M.
• Erythema necroticans migrans
• Erythema elevatum diutinum
• Impetigo herpetiformis
• Small vessel vasculitis(leukocytoclastic vasculitis)
• Dermatitis herpetiformis
• Linear IgA dermatosis
• Bacterid Andrews, pustular (pustulosis palmo-plantaris)
• Reiter's syndrome
• SAPHO syndrome

• Intestinal-associated dermatosis-arthritis syndrome

The pathogenesis of this clinically very different group of diseases has not yet been clarified. However, it is likely that they are not uniform but polyetiological - autoimmunological, vasculitic, infectious-allergic - in nature.

It can be assumed that all neutrophilic dermatoses are autoinflammatory in nature with predominant congenital immune dysfunction. Inflammatory mediators such as interleukin-1beta, TNF-alpha, interleukin-8, interleukin-17 and interleukin-36, which ultimately promote the activation and migration of neutrophils into the skin, are overexpressed. The occurrence of neutrophilic dermatoses in connection with a so-called "paradoxical skin reaction" as a side effect of therapy with TNF-alpha inhibitors is noteworthy.

The skin symptoms vary considerably from clinical picture to clinical picture. They range from vesiculo-pustular to papular, plaque-like, discoid, anular or polycyclic lesions. The polymorphism of the clinical symptoms in one and the same patient is characteristic, and depending on the localization of the infiltrate, the skin changes can be classified as follows:

• Superficial neutrophilic dermatoses
• Plaque-like neutrophilic dermatoses
• Deep neutrophilic dermatoses.

A characteristic feature of neutrophilic dermatoses is the polymorphism of clinical symptoms in one and the same patient.

1. Calabrese L et al. (2024) Paradoxical skin reaction to certolizumab, an overlap of neutrophilic dermatoses. J Dtsch Dermatol Ges 22:438-441.

2. Kühl E (2010) Reactive neutrophilic dermatoses. Akt Dermatol 36: 165-170

3. Callen JP (2002) Neutrophilic dermatoses. Dermatol Clin 20: 409-419