Sweet’s syndrome is eponym for acute febrile neutrophilic dermatosis coined by Dr Sweet from Plymouth, England, who first described this condition in 1964. It is characterized by acute fever, leukocytosis; with sudden appearance of tender erythematous papules, nodules, and plaques. The edema is so intense that the papules and nodules often resemble vesicles so often the description used for the papule is pseudovesicle. The plaques are formed by coalition of papules that gives an arcuate appearance. Sweetâ€™s syndrome is common in middle-aged women, but men, children are also be affected. Hematological investigation shows neutrophilia with diffuse infiltrate of neutrophils in the middle and upper dermis of skin biopsy.
Sweetâ€™s syndrome is characterized by four features: tender erythematous plaques with arcuate border and the plaque studded with pseudovesicles. The other associated features are: fever; conjunctivitis, leukocytosis, and papillary dermal infiltration of neutrophils in the skin biopsy. Fever and neutrophilia is not a consistent feature. The diagnosis is mostly based on the typical skin eruption and characteristic histological finding in skin biopsy.
Based on the clinical setting the SS is classified into classical or idiopathic sweetâ€™s syndrome, malignancy-associated SS and Drug-induced Sweetâ€™s syndrome. Classical Sweet’s syndrome (CSS) usually presents in women between the ages of 30 to 50 years, it is often preceded by an upper respiratory tract infection and may be associated with inflammatory bowel disease and pregnancy. The malignancy-associated Sweet’s syndrome (MASS) can occur as a paraneoplastic syndrome in patients with an established cancer or individuals, whose Sweet’s syndrome is related hematologic dyscrasia in malignant associated SS. The dermatosis in MASS may precede, follow, or appear concurrently with the diagnosis of malignancy. The most commonly associated malignancy is acute myelogenous leukemia and approximately 20% of cases are associated with hematogenous malignancy. Drug-induced Sweet’s syndrome (DISS) most commonly occurs in patients who are receiving granulocyte-colony stimulating factor, hematopoietic growth factor and the lesions typically occur when the patient has leukocytosis and neutrophilia. SS may precede 3 months to 6 years before the manifestation of any associated disease or malignancy, so these patients needs to kept under prolonged follow up and regular clinical and hematological evaluation to know the early development of these condition. Because of this, it is believed to be reactive phenomenon or cutaneous marker of systemic diseases. At the same time, it is also suggested to be having genetic association but no genetic link has been identified till today.
The skin lesions predominantly occurs on the head, neck, legs, and arms, and particularly on the fore arm and cheeks. Development of fever (50%); arthralgia or arthritis (62%); eye involvement in the form of conjunctivitis or iridocyclitis (38%); and oral aphthus (13%) has been noted. Other organs affected are: bones, nervous system, kidneys, intestines, liver, heart, lungs, muscles and spleen. Rarely sweetâ€™s syndrome lesions may turn bluish or grey and form abscess-like nodule and ulcerates known as â€˜pustular vasculitis.
Hematological investigations of these patients frequently shows neutrophilia (in less than 50%), elevated ESR (greater than 30â€‰mm/hr) (in 90%), and a slight increase in alkaline phosphatase (in 83%) cases, C-reactive protein (CRP); and p-ANCA (antineutrophil cytoplasmic antibody) is some. Skin biopsy shows mixed infiltrate of polymorphonuclear cells with nuclear fragmentation and histiocytes in the mid and papillary dermis of skin biopsy. The infiltrate is predominantly perivascular with swollen endothelial cell, and vessels, however, changes like: thrombosis; fibrin deposits, complement, or immunoglobulins are seen within the vessel walls; red blood cell extravasation ; inflammatory infiltration of vessel walls are absent in early lesions, unlike vasculitis. But, the features of vasculitis do not exclude the diagnosis of SS.
Sweets syndrome responds to systemic corticosteroids given at the dose of 0.5 to 1.5â€‰mg/kg of body weight per day. It cases rapid improvement of the symptoms and skin lesions, so itâ€™s regarded as the â€œgold standardâ€ in the treatment for SS. The skin eruption, increase nerutrophil count and fever improve within 72 hours of treatment with corticosteroid. The complete clearance of skin lesions takes about 3 to 9 days, however, frequent recurrences is seen in one-third cases. Corticosteroids are tapered over 2 to 6 weeks. The disease may clear spontaneously in some patients. Other drug effective is oral potassium iodide, colchicines. dapsone, doycycline, clofazimineand and cyclosporine. These drugs are indicated, when the use of steroid is contraindicated. Our cases were given corticosteroid with complete clearance of their lesions with no relapse after discontinuation of corticosteroid.
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