A 59y.o. male presents after a fall with pain in the hand

hint the “rash” first occurred two weeks before the fall.

sweets.JPG

Our patient had Sweet’s syndrome or acute febrile neutrophilic dermatosis. .  It is a constellation of clinical symptoms and physical features including a skin rash with neutrophils in the upper dermis and elevated WBC. Vasculitis can occur secondary to noxious products released from neutrophils. The edema in the dermis can be so severe that bullae develop The syndrome was first described by Dr. Robert Sweet in 1964 in the British Journal of Dermatology.

chest lesions in our patient

chest lesions in our patient

There are several causes Of Sweet’s syndrome including: malignancy, drug-induced and idiopathic( which are the most common). The most common malignancies are hematologic.  It may be preceded by a URI or associated with pregnancy or inflammatory bowel disease. In drug induced forms; colony stimulating factor  is a common cause.

causes of Sweet’s syndrome are varied.

causes of Sweet’s syndrome are varied.

While the most common site of neutrophil infiltration is the skin; bones , ears, eyes, kidneys, liver , heart and spleen have all been reported as sites of Sweet’s syndrome. The lesions can be confused with sporotrichosis, atypical tuberculosis, pyoderma gangrenosum or vasculitis which is why a biopsy must be done. Often in the ED they are treated as infection.

 

another pt seen in the ED with Sweet’s syndrome and treated erroneously for weeks as infection

another pt seen in the ED with Sweet’s syndrome and treated erroneously for weeks as infection

Since Sweet’s is thought to be a hypersensitivity reaction and is cytokine mediated, steroids are the gold standard for treatment. Colchicine and potassium iodide have also been used.

In the case of our patient the cause of the Sweet’s has not been determined but hep C and cocaine are both noted to cause it. He developed the rash on his chest prior to falling on the floor.  The necrosis of the hand was thought due to vasculitis probably from toxic products released from neutrophils and aggravated by   lying on the hand five days. The lesions on the chest were characteristic of Sweet’s syndrome and responded to steroids.  Plastics is following the necrotic fingers and partial amputations are planned.  The pt initially had blood cultures + for MSSA and strep mitis.  His TEE was negative for any source of emboli. 

Cohen PR, Kurzrock R. Sweet’s syndrome revisited: a review of disease concepts. Int J Dermatol 2003;42:761-778.

Baer YS, Salter SA. Acute Neurtrophilic Dermatosis. Emedicine Journal. June 18 2015. Avaiable at : http://emedicine.medscape.com/article/1122152-overview

Malone J, Slone S, Wills-Frank L, et al. Vascular inflammation(Vasculitis) in Sweet syndrome. 2002 Arch Dermatol. 138(3):345-49. Doi-10