Transcript Sweet syndrome

SWEET`S SYNDROME
ACUTE FEBRILE NEUTROPHILIC
DERMATOSIS
감염내과 R4 정명화
INTRODUCTION
Described by Dr. Robert Douglas Sweet in 1964
von den Driesch P: Sweet's syndrome (acute febrile neutrophilic dermatosis). J Am Acad Dermatol 1994, 31:535-556.
PATHOPHYSIOLOGY
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Bacterial antigen injected  skin lesion occurred
: hypersensitivity to Ag-Ab immune complex
Petrozzi JW, Warthan TL. Sweet’s syndrome: unique local response
to streptococcal antigen. Cutis 1976;17:267-72.
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Inappropriated activation IL-1,3,6,8, G-CSF, GM -CSF
von den Driesch P. Sweet’s syndrome (acute febrile neutrophilic
dermatosis). J Am Acad Dermatol 1994;31:535-56.
Cohen PR, Almeida L, Kurzrock R: Acute febrile neutrophilic
dermatosis. Am Fam Physician 1989;39(3)
Cancer
Hematologic malignancy (M/C
AML), solid tumor (commonly
GU,GI, breast ca.)
Infection
URI (streptococcus), GI infection
(salmonellosis, yersiniosis)
Inflammatory bowel
disease
Crohn`s disease, ulcerative colitis
Medication
Pregnancy
HISTOPATHOLOGY
Classical Sweet`s syndrome
Drug-induced Sweet's syndrome
Malignancy –associated Sweet`s syndrome
Drug induced sweet`s
Malignancy –associated Sweet`s syndrome
- 21% had a malignancy in Sweet`s disease.
- Most often associated with AML
- Carcinoma of genitourinary organ, breast, GI tract are most frequently
occuring cancer.
Haverstock C, Libecco JF, Sadeghi P, Maytin E: Tender erythematous
plaques in a woman with acute myelogenous leukemia.
Arch Dermatol 2006, 142:235-240.
EXTRACUTANEOUS MANIFESTATION
Cohen PR, Kurzrock R: Sweet's syndrome revisited: a review of disease concepts
. Int J Dermatol 2003;42:761–778
VARIANT OF SWEET`S SYNDROME
- PUSTULAR VASCULITIS
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Pustular lesion that occur on purpuric base
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Neutrophilic vascular reaction ~ fully developed
leukocytoclastic vasculitis.
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Located usually in dorsal hand
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Encompassed patients in whom pustular eruption of UC
- 1. Febrile illness
2. Neutrophil leukocytosis
3. Predominance of female patient
4. Failure to respond to antibiotics
5. Rapid resolution with prednisolone therapy
DIFFERENTIAL DIAGNOSIS
Cutaneous
Rosacea fulminans. Halogenoderma
Acute generalized exanthematous pustulosis
Pustular contact dermatitis, Generalized psoriasis
Infectious
Bacterial sepsis, cellulitis, erysipelas, Herpes simplex
Herpes zoster virus, Leprosy, Lymphagitis, panniculitis ,
Pyoderma gangrenosum, syphilis, Systemic mycosis,
Thrombophlebitis, Tuberculosis, Viral exanthem
Bacterial folliculitis, Furunculosis, Impetigo, Staphylococcus
scaled skin syndrome , Dermatophyte
Neoplastic
Chloroma, Lekemia cutis, Lymphoma, Metastatic cancer
Systemic
disease
Behcet`s disease, Bowel bypass syndrome,
Dermatomyositis, Familial Mediterranean fever, SLE
Vasculitis
Erythema elevatum diutinum, Granuloma faciale
Leukocytoclastic vasculitis, Periarteritis nodosa.
AGEP
(ACUTE GENERALIZED
EXANTHEMATOUS PUSTULOSIS)
- Rapid development of a febrile pustular eruption a few
hours or days after beginning a drug treatment
- Clinical finding of dozens to hundreds of pinhead-sized,
nonfollicular pustules on a background of edematous
erythema
- Leukocytosis with marked neutrophilia (>7000/microL)
- Pustular smear and culture negative for bacteria
- Rapid resolution of the rash after drug discontinuation
VIRAL EXANTHEM
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Widespread rash
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Particuarly affected children
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Parvoirus B19, dengue and yellow fever, west Nile
Marmah Forest, Marburg, Ebola virus, Human herpesvirus
Measle, rubella,mumps, enteroviruses, Lassa fever
Carneiro SC, Cestari T, Allen SH, Ramos e-Silva M. Viral exanthems in the tropics.
Clin Dermatol. 2007 Mar-Apr;25(2):212-20.
TREATMENT
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Cure or remission of related cancer
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Stopping the associated medication
TREATMENT
Systemic
corticosteroid
pulse
1mg/kg/day , within 4-6 weeks, taper dose to
10mg/day, some patient require 2-3 months to treat
up to 1g/day for 3-5 days
Topical steoroid
Potassium iodide
300mg three times each day
Colchicine
0.5mg three times each day
TREATMENT
Indomethacin
150mg once daily for 7days, then 100mg/ day for 14d.
Clofazimine
200mg once daily for 4 weeks, then 100mg for 4 weeks
Cyclosporin
2-10mg/kg/day every 2 days and discontinued on day 21
Dapsone
100-200mg/day
PROGNOSIS
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The cutaneous lesions of Sweet syndrome usually heal
without scarring. However, postinflammatory
hyperpigmentation that takes months
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Relapse after discontinuation of systemic glucocorticoids or
other therapies and may be more likely to occur in patients
with malignancy
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Recurrences : 30 percent of patients with classical Sweet `s
syndrome VS 69 percent underlying hematologic
malignancy