Sweet's Syndrome

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Contents 1 Introduction 2 Etiology 3 Epidemiology 4 Pathology 5 Clinical-manifestations 6 Laboratory 7 Differential-diagnosis 8 Management 9 More General Terms 10 Additional Terms 11 References

Introduction

• Uncommon, recurrent skin disease characterized by painful inflammatory papules & plaques associated with fever, arthralgia & leukocytosis.

Etiology

• idiopathic
• possibly hypersensitivity reaction
• some cases associated with Yersinia infection
• associated disorders

• febrile upper respiratory tract infection
• hematopoietic disorders (most common)

• acute myeloid leukemia ( AML)
• myelodysplastic syndrome
• transient myeloid proliferation

• other malignant tumors

• breast cancer
• gastrointestinal cancer
• urogenital neoplasms

• benign monoclonal gammopathy
• ulcerative colitis

• pharmaceutical agents

• G-CSF, all trans retinoic acid, Bactrim, hydralazine, lithium carbonate, furosemide, minocycline, BCG vaccine

• pregnancy (1st or 2nd trimester)

Epidemiology

• majority of patients are 30-60 years of age
• females > males
• rare disorder

Pathology

• epidermis generally normal, but may show subcorneal pustulation
• massive edema of papillary dermis
• dense leukocytic infiltrate of lower dermis

• diffuse or perivascular
• neutrophils with occasional eosinophils &/or lymphocytes
• leukocytoclasia leading to nuclear dust

• may be neutrophilic infiltrates in subcutaneous tissues

Clinical-manifestations

• prodome 1-3 weeks prior to skin lesions

• febrile upper respiratory tract infection
• diarrhea
• tonsillitis
• flu-like syndrome

• systemic manifestations may precede skin lesions

• fever
• headache
• arthralgia, myalgia
• malaise

• skin lesions

• papules or nodules that coalesce to form irregular, sharply demarcated inflammatory plaques
• intense edema gives the appearance of vesiculation
• tiny pustules many or may not be present
• bullous lesions may occur if associated with leukemia
• lesions red or blueish red in color
• lesions are tender or painful
• central clearing may lead to annular appearance
• single or multiple lesions distributed asymmetrically
• lesions grow rapidly & may become blisters that burn or itch
• distribution

• most commonly: face, neck & upper extremities
• lower extremities
• upper trunk
• generalized form may occur

• pathergy: development of lesions in areas of minor trauma
• lesions heal without scarring

• lesions of the oral mucosa
• eye: conjunctivitis/ episcleritis
• lungs: alveolitis
• bone: sterile osteomyelitis
• involvement of liver, mucsle, pancreas, cardiovascular system & CNS may occur

Laboratory

• complete blood count ( CBC)

• neutrophilia
• leukocytosis may be absent in patients with underlying hemotologic malignancy

• elevated erythrocyte sedimentation rate (ESR)
• blood cultures to rule out sepsis
• skin biopsy

Differential-diagnosis

• erythema multiforme
• erythema nodosum
• prevesicular Herpes simplex
• pyoderma gangrenosum

• may occur in conjection with pyoderma gangrenosum

• bowel-bypass syndrome
• urticaria
• serum sickness
• SLE panniculitis
• other vasculitides

Management

• rule out sepsis
• prednisone

• 30-60 mg PO QD
• taper in 2-3 weeks

• dapsone 100 mg QD
• KI, colchicine, NSAIDs, clofazamine, pentoxifylline
• steroid-sparing agent for chronic or severe disease

• cyclosporine
• mycophenolate
• TNF-alpha inhibitor

• antibiotic therapy for Yersinia infection

• useless for other forms of Sweet's syndrome

• prognosis

• untreated lesions evolve over a period of days to weeks & eventually resolve without scarring after weeks to months
• lesions treated with prednisone resolve within a few days
• recurrences occur in 50% of patients

More General Terms

• skin disease (dermatologic disorder, dermatopathy, dermatosis)
• syndrome

Additional Terms

• erythema multiforme
• erythema nodosum (septal panniculitis)
• Herpes simplex (HSV) or Herpes hominis
• hypersensitivity
• panniculitis
• pyoderma gangrenosum
• serum sickness
• urticaria (hives)
• Yersinia

References

1. Color Atlas & Synopsis of Clinical Dermatology, Common & Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 398-400
2. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 95
3. Medical Knowledge Self Assessment Program (MKSAP) 14, 15, American College of Physicians, Philadelphia 2006, 2009

Sweet's syndrome (acute febrile neutrophilic dermatosis)