Acute febrile neutrophilic dermatosis in Adult
It may be seen in patients of all ages, but it is most common in healthy women aged 20-60 and individuals with inflammatory bowel disease (IBD) or hematologic malignancies (especially myeloid leukemias and myelodysplastic syndrome). Other common associations include pregnancy, streptococcal pneumonia, autoimmune disorders, radiation therapy, lymphedema, and medications (sulfamethoxazole-trimethoprim, minocycline, granulocyte colony-stimulating factor). Most cases are idiopathic or associated with benign conditions; about 15%-20% are associated with malignancy.
Although the exact etiology is still unclear, abnormal cytokine expression and atypical neutrophil function are thought to contribute to the pathogenesis. A genetic predisposition may also be operable.
Pathergy is frequently associated, as lesions will arise or worsen in sites of cutaneous injury, such as needle sticks. Extracutaneous manifestations such as fever, headaches, myalgias, malaise, arthralgias, and ocular inflammation may be seen. Other sites that may be affected include the oral mucosa, gastrointestinal tract, musculoskeletal system, lungs, kidneys, heart, and central nervous system (CNS). Hypotension and tachycardia are rare but can occur as a result of systemic inflammation.
Neutrophilic dermatosis of the dorsal hands is considered by many to represent a localized form of Sweet syndrome. Hematologic malignancies and IBD are the most common associations with this subtype.
Necrotizing neutrophilic dermatosis describes a subset of patients with severe Sweet syndrome or pyoderma gangrenosum who develop, in addition to their cutaneous disease, fever, leukocytosis (or a leukemoid reaction), and features of shock. Skin pain is a prominent symptom. Additionally, soft tissues underlying areas of skin involvement may be affected with neutrophilic infiltrates and necrosis.
Sweet syndrome typically responds dramatically to systemic corticosteroids, but recurrences are common.
L98.2 – Febrile neutrophilic dermatosis [Sweet]
84625002 – Acute febrile neutrophilic dermatosis
- Pyoderma gangrenosum – Begins as a pustule and evolves into a purulent ulcer with rolled borders. Has similar associations, including IBD and malignancy.
- Neutrophilic eccrine hidradenitis – Drug-induced toxicity of the eccrine coils 1-2 weeks following chemotherapy. Palmar involvement is more suggestive of this diagnosis. Patients typically lack fevers but pathology may be required to differentiate these entities.
- Rheumatoid neutrophilic dermatosis
- Bowel-associated dermatosis-arthritis syndrome – Pustular skin lesions and aseptic arthritis in bowel-bypass patients.
- Wells syndrome – Involved skin is typically neither tender nor warm. Eosinophilia present in 50%, neutrophilia would be unusual. Eosinophils and flame figures seen on pathology.
- Necrotizing fasciitis – Necrotizing neutrophilic dermatosis may mimic necrotizing fasciitis. Histopathologic findings and blood and tissue cultures are useful diagnostically.
- Erythema multiforme – Should display classic target lesions with 3 zones of color, favors acral surfaces. Oral involvement common.
- Azathioprine hypersensitivity syndrome – Acute neutrophilic and systemic reaction after initiating azathioprine.
- Urticarial vasculitis
- Erythema elevatum diutinum – Primarily over extensor surfaces.
- Cutaneous small vessel vasculitis – Presents symmetrically on lower extremities, typically smaller than the plaques of Sweet syndrome.
- Behçet disease – Rare, recurrent, and associated with oral or genital ulcers.
- Bromoderma or iododerma
- Bacterial infections (furunculosis, cellulitis)
- Mycobacteria sp. infections (typical and atypical), including Mycobacterium marinum
- Erythema nodosum – Primarily over the shins.
- Leishmaniasis (New World and Old World) – Recent travel to endemic areas.
- Lymphoma / leukemia cutis
- Metastatic carcinoma
- Majocchi granuloma
- Cat-scratch disease
- Cutaneous anthrax
- VEXAS syndrome