Daniela Malieni

Síndrome de Sweet asociado a neoplasias

Sweets syndrome was described in 1964 by Robert Douglas Sweet, as an entity he named acute febrile neutrophilic dermatosis. It is characterized by five main features: 1) sudden appearance of erythematous and tender plaques on the face, neck and extremities; 2) fever; 3) polymorphonuclear leukocytes; 4) predominantly neutrophilic dense infiltrate in the dermis, and 5) rapid response to steroid therapy. Sweets syndrome can be classified into five groups: idiopathic, parainflammatory, paraneoplastic, drug-induced, and pregnancy-related. Twenty percent of cases are associated with malignancies; 85% out of them involve hematologic alignancies and the remaining 15%, solid tumors. A series of seven cases of Sweets syndrome associated with neoplasms which were diagnosed from 2002 to 2006 is presented. Six cases were related to oncohematologic diseases and one to solid tumors. These results highlight the importance of the diagnosis of the syndrome, since it may predict tumor relapse or underlying disease progression. The timely use of diagnostic and treatment methods may improve the quality of life of these patients. The fact that oncology patients take multiple medications (a colony-stimulating factor) which may be associated with the onset of this entity must also be considered in excluding possible causes.

Nephrogenic fibrosing dermopathy

We report a 19-year-old man with end-stage renal disease undergoing haemodialysis. He suffered from familial HUS and had been on haemodialysis since January 2004. After a corrective angioplastia of his left arm arteriovenous fistulae, he developed fever, oedema and limited mobility of extremities as well as myalgias. He had previously undergone magnetic resonance imaging with gadolinium containing contrast agents. An initial laboratory workup showed anaemia, hyperkalemia, hypercalcemia, hyperphosphatemia and increased ESR; bacterial, fungal and viral infections were ruled out. Due to severe and persistent hypercalcemia, pamidronate was administered. Calcium levels remained within normal limits. On physical examination, he presented global muscle atrophy with stiffness and flexion contractures in knees, hips and elbows. He had significant functional limitations and became unable to walk. The skin was thickened and indurate with brownish-grey, 4-mm-diameter, confluent, round papules with slightly desquamate area in lower extremities (fig. 1) and arms. A punch biopsy of the skin was done and showed dermal hyalinization and fibrosis with thickened collagen bundles (fig. 2). The immunohistochemical analysis showed the presence of numerous spindle CD 34 cells in dermis. All these findings were consistent with NFD. He was treated with methylprednisone 40 mg/day, anti–tumour necrosis factor monoclonal antibodies (etanercept) and 2 g/day mycophenolate mofetil. Muscle and joint pain disappeared, and flexion stiffness and skin lesions partially resolved. To our knowledge, this is the first case of familial HUS-associated NFD described in literature. The pathogenesis of NFD is still poorly understood. 1,3,4