A 45 y.o. construction worker states he has an infection in his hand. His hand is pictured.

 What is wrong?

What is wrong?

Our patient had Sweet’s disease or acute febrile neutrophilic dermatosis.  It is characterized by tender red, papules and plaques that show dense infiltrates of neutrophils.  It was first described by Robert Sweet in 1964 and four criteria have to be met to diagnose the disease: fever, leukocytosis, tender red plaques, and a dermal infiltrate of neutrophils. Often patients with the disease are treated several times with antibiotics for cellulitis/abscess and they do not improve because the treatment is steroids. In addition to the raised plaques pts present with fever in 50% ofcases,  arthritis in 62% of cases and eye findings in 38% of cases.

 skin biopsy of a pt with sweets showing neutrophils

skin biopsy of a pt with sweets showing neutrophils

It is classified by the setting in which it occurs either malignancy associated,  drug induced or idiopathic.

20% of the cases are associated with malignancy. It can also be associated with strep infections, inflammatory bowel disease, rheumatoid arthritis,  and pregnancy. Drugs that cause it include granulocyte colony-stimulating factor and granulocyte-macrophage colony-stimulating factor.  It is thought to represent a hyperactive immune response in response to a systemic process.   Steroids dramatically improve the condition in 72 hours presumably suppressing dysregulated cytokines.

The differential of Sweet’s includes:  pyodermagangranosum which appears as purple colored ulcers,  erythema multiforme which appears as red spots on the hands, feet and face( often in rings) and erythema nodosum which appears as raised plaques.

 pyoderma gangranosum in the setting of crohns

pyoderma gangranosum in the setting of crohns

The process usually lasts 2-4 weeks and can recur in one third of the patients.  In our patient, treatment with steroids caused resolution of the rash and he has not had a recurrence.

 

Mustafa MN, Lavizzo M. Sweet’s syndrome in a patient with Crohn’s disease: a case report.  J Med Case Reports 2008 2:221.

Cohen PR. Sweet’s syndrome-a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet Journal of Rare Diseases 2(34).

Sweet RD An acute febrile neutrophilic dermatosis. Br J Dermatol. 1964 76:349-56.