Sweets syndrome

To the Editor

Among the group of cutaneous vasculitis, acute febrile neutrophilic dermatoses (Sweets syndrome) is a relatively rare condition. The definition has remained relatively constant with four cardinal features; fever, peripheral luecocytosis, tender erythematous plaques on the limbs, face and neck with a tense dermal infiltrate of mature nuetrophils. There is a relationship with preceding infection, erythema multiforme, pyoderma gangrenosum, an association with myeloproliferative disorders (upto 50%). Herewith we are reporting a case of Sweets syndrome.

A 48-year-old lady reporter with complaints of recurrent skin lesions on the extremities, with similar lesions on the lips since 8 years. There was an associated history of mild fever, arthralgia of the knee joints, generalized weakness and inability to do household work with no relevant relation to drug intake. General examination showed pallor, platonychia, with minimal pedal edema. There was exacerbation of oral lesions following which she developed lesions on the extremities, which were tender erythematous to violaceous plaques and nodules affecting the dorsal aspect of extremities. Smaller annular plaques with peripheral vesicular lesions were also seen in the area.

On investigation the hemoglobin was only 6.3 gm%, ESR at 70mm 1st hour, luecocytosis, peripheral smear showing microcytic hypochromic anemia but she was unwilling for a bone marrow examination. Platelets were 6.51 lakhs. Mantoux was negative, RA factor, LE cell and ANA/ANCA were negative. A skin biopsy of the area showed prominent blood vessels with perivascular aggregates of mononuclear inflammatory cells mainly nuetrophils suggestive of a neutrophilic dermatosis. She had good response to (40mg) steroid with complete resolution of the lesions in 2 weeks and is being followed up presently since 6 months.

Sweets syndrome is a neutrophilic dermatoses which has a diagnostic criteria of abrupt onset tender painful erythematous or violaceous plaques or nodules, a neutrophilic infiltrate in the dermis without leucocytic vasculitis. Few minor criteria elucidated were fever preceding infection, arthralgia, conjunctivitis, a possible underlying malignancy, luekocytosis, and a good response to steroids. Our patient could be fixed into Sweets syndrome following this criteria with no relation to malignancy though severe anemia was noted. This case has been reported because of its rarity so that the consulting physician can keep this in mind as a differential diagnosis among the several similar conditions.