follow @PCDSUKCreated: 7th January 2015 | Last Updated: 23rd February 2015
Introduction
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This chapter provides a brief overview of the neutrophilic dermatoses, and is set out as below. For more information refer to the related chapters. |
Clinical findings
Common features found in the neutrophilic dermatoses are:
- Histological features dominated by a neutrophilic vascular reaction, typically falling short of a vasculitis
- A significant proportion have a reactive or underlying systemic association - mainly haematological, gastrointestinal, rheumatological or drug-induced
- The conditions may coexist
- The neutrophilic process itself may cause systemic illness
- They may arise at the site of injury such as a needle prick, biopsy or insect bite. This reaction is known as pathergy
The following is a list of the better-known types of neutrophilic dermatoses, it is by no means comprehensive:
- Pyoderma gangrenosum
- Sweet's syndrome (syn. acute febrile neutrophilic dermatoses)
- Possible variants of Sweet's eg neutrophilic dermatoses of the dorsal hands
- Subcorneal pustular dermatosis
- Neutrophilic panniculitis
- Relapsing polychondritis
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Conditions overlapping with vasculitis:
- Behçet's disease
- Erythema elevatum diutinum
- Specific links with gastrointestinal disease eg pustular vasculitis/neutrophilic pustulosis (many cases represent pustular pyoderma gangrenosum), aseptic abscess in Crohn's disease, peristomal pyoderma gangrenosum
- Associated with connective tissue disease eg rheumatoid neutrophilic dermatosis
- Associated with acne eg pyogenic arthritis with pyoderma gangrenosum and acne (PAPA)
- Paraneoplastic or typically chemotherapy-associated eg paraneoplastic neutrophilic figurate erythema
- Systemic neutrophilic dermatoses eg Neuro-Sweet's syndrome, or affecting other internal organs and causing, for example, myositis, pericarditis or pulmonary complications
- Familial Mediterranean fever