The Clinical Significance of a Pathergy Reaction in Patients with Behcet's Disease

This study was done to evaluate the frequency, intensity, and specificity of a positive pathergy reaction (PR) in Behcet's disease (BD) patients, to clarify an association between the PR and the clinical features or disease severity, and to assess whether patients with pustule formation at the venous puncture site (PFVPS) without positive PR could be regarded as a positive reaction. The PR was tested in 64 BD patients, 74 disease controls, and 20 healthy controls. Venous PR was performed in 8 BD patients with PFVPS. Follow-up PR was done in 14 patients with positive reaction during inactive phase. The PR was positive in 35.9% of BD patients, in 1 patient among disease controls, and in none of healthy controls. The pustule formation was observed in one BD patient. There was no statistical significance between positive PR and the clinical variables. The mean clinical activity score of BD patients with positive PR was similar to patients with negative reaction. Venous PR was positive in 7 patients. The follow-up PR was positive in 2 patients during inactive phase. Conclusively the positive PR appeared to be specific for BD, and was not associated with the clinical variables or disease severity, but was usually found during active phase in cases with positive reaction. The PFVPS in patients with negative PR might be considered to be positive.

An Unusual Case of Sweet's Syndrome Associated with Myelodysplastic Syndrome / 대한혈액학회지

Sweet's syndrome, initially described in 1964 as acute febrile neutrophilic dermatosis by Sweet, is characterized by five cardinal feature: fever; neutrophilia; abrupt appearance of erythematous, painful, cutaneous plaque, primarily located on the upper extremities, head and neck; a dermal infiltrate of mature neutrophils; and a rapid response to steroid therapy. More than 500 cases of Sweet's syndrome have been documented since original description of Sweet, of which, approximately, 15~20 percent of published cases occurred in patient with hematologic malignancy. The authors describe an unusual case of Sweet's syndrome in a man, who affected with myelodysplastic syndrome (RAEB). A 56-year-old male patient admitted to Yonsei University, Medical Center because of sustained fever and cellulitis of right lower leg. With anemia and thrombocytopenia, the bone marrow study revealed myelodysplastic syndrome (RAEB). On 4th hospital day, with the development of pneumonia, he was treated with mechanical ventilation and broad spectrum of antibiotics. Although the treatment of pneumonia was successful, erythematous nodules, showing Koebner phenomenon and strong positive pathergy reaction, was appeared on the whole body (21st hospital day). Skin biopsy was taken. Under the diagnosis of Sweet's syndrome associated with myelodysplastic syndrome (RAEB), oral prednisolone 60mg/day was prescribed. The cuteneous lesions were regressed, but the brownish pigmentation was remained, till the patient discharged.