A 35 yr old female presented with fever, malaise, leukoocytocis and painful indurated plaques on forearm and hand. Name the syndrome ? Hint : This syndrome is associated with internal malignancy in approximately 20% of cases, most notably AML
Answer: Sweet syndrome Discussion Sweet’s syndrome occurs mostly in women 30 to 60 years of age and consists of characteristic skin lesions, fever, malaise, and leukocytosis. Less commonly, there is involvement of the joints, eyes, lungs, kidneys, and liver. Approximately 20% of cases have an association with a hematopoietic malignancy or, more rarely, a solid tumor. The clinical hallmark of Sweet’s syndrome is the presence of sharply demarcated, painful plaques on the face, neck, upper trunk, and extremities (see fig above ). The surface of the plaques has a mammillated (nipple-like) appearance and often shows papulovesicles and pustules. Some lesions have a target-like appearance, and lesions on the lower extremities may resemble erythema nodosum. Oral mucous membrane and eye lesions can be seen. Skin lesions may develop at the site of minor skin trauma or needle-sticks in a small subset of patients. This phenomenon is called pathergy and is also seen in pyoderma gangrenosum and Behçet’s syndrome. Lab findings Leukocytosis. Ten thousand/mm3 is present in 60% of patients. Elevated sedimentation rates, increased numbers of segmented neutrophils, lymphopenia, anemia, thrombocytopenia, and increased C-reactive protein levels can be seen. A handful of cases have been reported with antineutrophilic cytoplasmic antibodies. The most commonly associated malignancy is acute myelogenous leukemia, but chronic myelogenous leukemia, lymphocytic leukemia, T- and B-cell lymphomas, polycythemia, and, rarely, solid tumors also have been reported. There are no clinical or histopathologic differences between patients with and without associated cancer. Patients with persistent laboratory abnormalities, especially anemia, thrombocytosis, and thrombocytopenia, require close observation and thorough diagnostic evaluation. Credit to Chriss for clinching the diagnosis.
