Sweet's syndrome in a woman with a prothrombin gene (G20210A) mutation

Abstract

A 46-year-old woman was admitted to our hospital with tender, erythematous plaques on her palms of 1-year duration (Fig. 1). She had a history of fever and upper respiratory tract infection. On dermatological examination, there were found to be tender erythematous papules and plaques on the palms. Physical examination was normal. Ophthalmologic examination revealed bilateral episcleritis. Laboratory tests showed the following values: white blood cell count, 15,600 cells/µL with 3% band forms, 74% segmented neutrophils, 3% monocytes, and 20% lymphocytes; red blood cell count, 4.53 × 106 cells/µL; hemoglobin, 13.2 g/dL; hematocrit value, 40.9%, and platelet count, 241 × 109 platelets/µL. The erythrocyte sedimentation rate was 52 mm/ h, and the C-reactive protein level was 38.8 mg/dL. Cultures from blood and throat swabs for bacteria revealed no pathogenic growths. Urinanalysis, liver and kidney function tests were within normal limits. Antinuclear, anti-DNA, and antiphospholipid antibodies were negative, and total C3 and C4 complement levels were normal. In histopathological examination, large infiltrates of neutrophils and nuclear dust were seen in a diffuse pattern within the edematous dermis. There were scattered neutrophils within the epidermis. The vascular endothelial cells were plump, but there was no fibrin in the wall of the venules (Figs 2 and 3). In addition, there was a heterozygous prothrombin (G20210A) gene mutation in rapid polymerase chain reaction (PCR) analysis. She had no history of venous thrombosis or hematologic disorders. The following coagulation and thrombophilic tests were normal: activated protein C ratio (2.6; normal > 2), protein C (84%; normal: 78–106%), prothrombin time (9.8 s; normal: 7–13 s), activated partial thromboplastin time (29.6 s; normal: 22.6–35.0 s), fibrinogen (312 mg/dL; normal: 146–400 mg/dL), bleeding time (30 s, Duke method), and clotting time (5 min, Lee-White method). Chest X-ray, abdominal ultrasound findings and tumor marker levels were normal. Based on clinical, laboratory and histopathological findings a diagnosis of Sweet's syndrome associated with prothrombin gene mutation was made. The skin lesions resolved rapidly on treatment with wet compresses of Burrow's solution and oral prednisolone (1 mg/kg). The dose of prednisolone was gradually reduced, and was discontinued after 4 weeks. Figure 1. Erythematous plaques on the palmar aspect of the hand Download figure to PowerPoint Figure 2. Large infiltrates of neutrophils and nuclear dust of neutrophils were seen in a diffuse pattern in the dermis (hematoxylin and eosin, ×65) Download figure to PowerPoint Figure 3. Higher magnification of Fig. 2 (hematoxylin and eosin, ×130) Download figure to PowerPoint