Sweet syndrome in Child
Sweet syndrome is a sterile neutrophilic disorder, and like its counterpart pyoderma gangrenosum, is associated with inflammatory bowel disease (IBD), streptococcal infections, rheumatoid arthritis, medications (typically G-CSF), and hematologic disorders, especially myeloid leukemias and myelodysplastic syndrome. In children, Sweet syndrome has also been reported in chronic granulomatous disease and periodic fever syndromes.
Fever may accompany or precede cutaneous involvement. The syndrome frequently includes extracutaneous manifestations such as fever, headaches, myalgias, malaise, arthralgias, and ocular inflammation. Other sites that may be affected include the oral mucosa, gastrointestinal (GI) tract, lungs, musculoskeletal system, kidneys, heart, and central nervous system (CNS). Hypotension and tachycardia are rare but can occur as a result of systemic inflammation.
Although the exact etiology is still unclear, Sweet syndrome has been considered a dysregulated cytokine response associated with inflammatory, infectious, and malignant disease. It demonstrates pathergy and will arise in sites of cutaneous injury, such as needle sticks.
Sweet syndrome typically responds dramatically to systemic corticosteroids, but recurrences are common.
L98.2 – Febrile neutrophilic dermatosis [Sweet]
84625002 – Sweet's syndrome
- Bacterial infections (furunculosis, cellulitis)
- Mycobacteria sp. infections (typical and atypical) including Mycobacterium marinum
- Majocchi granuloma
- Erythema nodosum – Primarily over the shins.
- Acute hemorrhagic edema of infancy
- Pyoderma gangrenosum – Begins as a pustule and evolves into a purulent ulcer with rolled borders. Has similar associations with 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.
- Erythema multiforme – Should display classic target lesions with 3 zones of color, favors acral surfaces. Oral involvement common.
- Bowel-associated dermatosis-arthritis syndrome – Pustular skin lesions and aseptic arthritis in bowel-bypass patients.
- Majeed syndrome – Multifocal osteomyelitis, congenital anemia, and Sweet-like skin dermatosis.
- Chronic granulomatous disease
- 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.
- Drug eruption
- 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.
- Behçet disease – Rare, recurrent, and associated with oral or genital ulcers.
- Bromoderma or iododerma
- Leishmaniasis (New World and Old World) – Recent travel to endemic areas.
- Lymphoma / leukemia cutis
- Metastatic carcinoma
- Cat-scratch disease
- Cutaneous anthrax
Last Updated: 01/16/2017