Diagnosis

Acute febrile neutrophilic dermatosis

Etiology

Etiology

• Unknown (50%)

• Leukemia (20%)

• Other malignancies

• Infection (Streptococcal pneumonia)

• Inflammation

• Vaccination

• Drug exposure (GM-CSF)

• Pregnancy

• Becets disease

Pathophysiology [ssf]

A neutrophil mediated immunological response (hypersensitivity reaction) to bacterial, viral, neoplastic, or other antigens (vaccine, drug, malignancy):

• Cutaneus disease (most common): Erythematous, tender, nonpruritic papules, nodules, and plaques with predominantly head, neck, and upper trunk distribution patterns

• Oral: Oral ulcers

• Eye: Ocular inflammation (conjunctivitis, episcleritis, scleritis, ulcerative keratitis, iritis, glaucoma, dacryoadenitis, and choroiditis)

• Musculoskeletal: Arthralgia, arthritis, and myalgia

• CNS: Encephalitis, menigitis

• Pulmonary system:  Alveolitis, pleural effusion, airway obstruction

• Cardiovascular system: Myocarditis, aortitis, coronary artery occlusion

• Liver: Hepatitis, hepatomegaly

• Intestines: Enteritis

• Spleen: Splenomegaly

• Kidneys: Glomerulonephritis, hematuria, proteinuria

• Bone: Sterile osteomyelitis

Epidemiology

Incidence per 100.000 [wtr][m4d][ssf]

Approach

Diagnostic criteria [ssf]

Major criteria

1. Rapid onset of tender erythematous plaques and nodules

2. Typical histopathology: dense neutrophil infiltration without leukocytoclastic vasculitis

Minor criteria

1. Fever (>38 ̊C)

2. Association with an underlying hematologic or visceral malignancy, inflammatory disease, or pregnancy, or preceded by an upper respiratory or gastrointestinal infection or vaccination

3. Excellent response to treatment with systemic corticosteroids or potassium iodide

4. Abnormal laboratory values at presentation (three of four):

   a) Erythrocyte sedimentation rate >20 mm/h

   b) High C-reactive protein

   c) WBC >8x109/l

   d) Neutrophil count >70% of total WBC count

The diagnosis relies on the presence of at least 3 of these factors. The presence of both major criteria (1 and 2) and two of the four minor criteria confirms the diagnosis of classical Sweet's syndrome

Treatment

1. Treatment of underlying cancer, infection, or inflammation [ssf]

2. Withdrawal of relevant drug

3. Systemic corticosteroid therapy

4. Other treatments: Colchicine, Dapsone, Potassium iodide, Indomethacin

Differential diagnoses

Behcet's disease, Bullous pemphigoid, Cellulitis, Crohn disease, Drug eruptions, Ecthyma, Eczema, Epidermolysis bullosa, Erysipelas, Erythema multiforme, Erythema nodosum, Fungal infection, Herpes simplex, Leukemia, Leukemia cutis, Linear IgA dermatosis, Lymphoma, Polyarteritis nodosa, Pyoderma gangrenosum, Sarcoidosis, Systemic lupus erythematosus, Urticaria, Urticarial vasculitis