Sweet's syndrome (SS) is a skin disease of unknown etiology1,2 which is characterized by fever, leukocytosis, and painful erythematous skin plaques. Histology shows dense neutrophilic infiltrates. The presence of anemia and thrombocytopenia may be associated with an underlying neoplastic.3

We present the case of a patient with SS and anemia diagnosed as a myelodysplastic syndrome (MDS), worsening the prognosis.

MDS are hematologic diseases characterized by cytopenias that result in dysmorphic states of the cells of the bone marrow. One type of MDS is refractory anemia with blast excess (RABE), with the number of blasts being greater than 5% and less than 10% in type 1, and between 10% and 20% in type 2. These diseases are refractory to chemotherapy and stem cell transplant offers a cure. In RABE, supportive treatment is performed with red blood cell transfusions.

The patient is a 70 years old male with no history of interest, who had recurrent episodes of 2 weeks with high fever, leukocytosis, and erythematous papules and plaques on the extremities, with dense neutrophilic infiltrates in the biopsy (Fig. 1). The patient had asymmetric oligoarthritis affecting the wrist and knees. Analytically, there was an elevated sedimentation rate (ESR), leukocytosis, and anemia. Episodes were treated with glucocorticoids at 0.5mg/kg, with little response. The disease became chronic and recurrent. Indomethacin was prescribed a dose of 150mg/day and potassium iodide was added, without improvement. In parallel, the patient was diagnosed with a type 1 RABE after the study of persistent anemia. We performed multiple red blood cells transfusions.

Fig. 1.

Sweet's syndrome: moderate edema of the papillary dermis and a dense neutrophil infiltrate of the reticular dermis.

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Patient outcome was not favorable, with persisting episodes of fever, and arthritis. His anemia worsened and underwent a bone marrow transplant. While waiting for a compatible donor, the patient died, possibly due to a complication of the hematologic process, worsening of cytopenias or transformation to leukemia.

SS can be classified into 3 groups: idiopathic, associated with malignant disease and drug-induced. Up to 54% of patients may have a tumor or hematologic disease.4 Joint involvement may occur (33%–62%), as well as lung, eye, kidney, liver, or central nervous system compromise. Chronic recurrent disease occurs in around 15%.1 The disease is more common in women in the fifth decade of life. The ESR is elevated and anemia present. It can last from 1 week to 4 years. The differential diagnosis must be made with erythema nodosum, cellulitis and erysipelas, erythema elevatum diutinum, erythema multiforme, leukocytoclastic vasculitis, or pyoderma gangrenosum.

Systemic glucocorticoids are the treatment of choice. Indomethacin5 is effective. Colchicine, potassium iodide, dapsone, cyclosporine, interferon alpha or etretinate6 are therapeutic alternatives. There have been reports of SS and MDS successfully treated with intravenous immunoglobulin,7 glucocorticoids, and doxycycline or stem cell transplantation.8,9

The patient described had a case that progressed despite treatment. Pending the transplantation we were able to use some of the treatments described in the literature, although possibly the transplant would had been the only effective treatment.

SS is a rare dermatological entity, which when accompanied by an MDS may worsen the patient's prognosis. The treatment of choice for SS is the use of glucocorticoids, although in most cases this may be insufficient and require alternative therapies.

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The authors have no disclosures to make.