Reactive neutrophilic dermatoses
| Reactive neutrophilic dermatoses | |
|---|---|
| Synonyms | |
| Pronounce | N/A |
| Specialty | Dermatology |
| Symptoms | Skin lesions, fever, arthralgia |
| Complications | N/A |
| Onset | |
| Duration | |
| Types | N/A |
| Causes | Immune system dysregulation |
| Risks | |
| Diagnosis | Clinical diagnosis, skin biopsy |
| Differential diagnosis | Infection, vasculitis, autoimmune disease |
| Prevention | N/A |
| Treatment | Corticosteroids, immunosuppressive therapy |
| Medication | |
| Prognosis | |
| Frequency | |
| Deaths | N/A |
Group of skin conditions characterized by neutrophilic infiltration
Reactive neutrophilic dermatoses are a group of skin conditions characterized by the presence of neutrophils in the dermis without evidence of infection. These conditions are often associated with systemic diseases and can present with a variety of skin lesions.
Classification
Reactive neutrophilic dermatoses can be classified into several distinct entities, each with unique clinical features:
Sweet's Syndrome
Sweet's syndrome, also known as acute febrile neutrophilic dermatosis, is characterized by the sudden onset of fever, leukocytosis, and tender erythematous skin plaques. It is often associated with underlying conditions such as malignancy, inflammatory bowel disease, or infection.
Pyoderma Gangrenosum
Pyoderma gangrenosum is a condition that presents with painful pustules or nodules that break down to form ulcers with undermined borders. It is frequently associated with systemic diseases such as ulcerative colitis, Crohn's disease, and rheumatoid arthritis.
Behçet's Disease
Behçet's disease is a systemic vasculitis that can cause recurrent oral and genital ulcers, uveitis, and skin lesions. The skin manifestations can include erythema nodosum-like lesions and papulopustular lesions.
Subcorneal Pustular Dermatosis
Subcorneal pustular dermatosis, also known as Sneddon-Wilkinson disease, is characterized by superficial pustules that primarily affect the trunk and intertriginous areas. It is considered a chronic relapsing condition.
Pathophysiology
The pathophysiology of reactive neutrophilic dermatoses involves the abnormal accumulation of neutrophils in the skin. This accumulation is thought to be due to an exaggerated immune response, often triggered by an underlying systemic condition. The exact mechanisms are not fully understood, but cytokines such as interleukin-1 and tumor necrosis factor-alpha are believed to play a role.
Diagnosis
Diagnosis of reactive neutrophilic dermatoses is primarily clinical, supported by histopathological examination of skin biopsies. The biopsy typically shows a dense neutrophilic infiltrate in the dermis without evidence of vasculitis. Laboratory tests may be conducted to identify any associated systemic conditions.
Treatment
Treatment of reactive neutrophilic dermatoses involves addressing the underlying systemic condition and managing the skin lesions. Systemic corticosteroids are often the first line of treatment. Other options include dapsone, colchicine, and immunosuppressive agents such as cyclosporine or azathioprine.
Prognosis
The prognosis of reactive neutrophilic dermatoses varies depending on the underlying condition and the response to treatment. Some patients may experience recurrent episodes, while others may achieve long-term remission.
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Contributors: Prab R. Tumpati, MD