ISSN:
1365-4632
Quelle:
Blackwell Publishing Journal Backfiles 1879-2005
Thema:
Medizin
Notizen:
A 32-year-old man, with a 12-year history of recurrent edema of the upper and lower extremities, was admitted in February 1998 due to edema of the right forearm and tiredness of 1-month duration. Examination revealed an ill-defined, itchy, slightly erythematous, indurated patch with excoriation on the right forearm (〈link href="#f1"〉Fig. 1). There was no history of drug intake, atopy, or asthma. He recalled, however, having experienced cold-induced Raynaud's phenomenon and intermittent arthralgia. In laboratory tests, there was a white cell count of 11.77 × 103/μL with 48% eosinophils (4830/μL). Liver function tests showed elevated serum glutamic pyruvic transaminase (SGPT) and alkaline phosphatase. Rheumatoid factor was positive. Total immunoglobulin E (IgE) concentration was elevated to 4703 U/mL (normal, 〈 378 U/mL), while other serum immunoglobulins, including IgG, IgA, and IgM, were normal. Bone marrow aspirate and biopsy showed normocellular marrow with an excess of mature eosinophils, but no evidence of blast cells. There was no evidence of parasitic infestation, and allergy skin test was negative. An extensive systemic evaluation showed no other organ involvement. A skin biopsy specimen revealed many eosinophils infiltrating the perivascular, periappendageal, and subcutaneous fat area and also within the vessel walls. Multiple microvascular thrombi with eosinophils were observed in the mid and lower dermis and subcutaneous fat (〈link href="#f2"〉Fig. 2). The eosinophils showed positive staining with eosinophil cationic protein (ECP) using the monoclonal antibody EG2. On the basis of the above findings, we diagnosed hypereosinophilic syndrome (HES). Treatment with 60 mg prednisolone daily was started and, within 7 days, his edema and itching sensation started to resolve. Follow-up laboratory and biopsy findings after 1 month returned to normal. The prednisolone dosage was slowly tapered over a 9-month period and he is currently on a maintenance dose of prednisolone 10 mg alternate day therapy.〈figure xml:id="f1"〉1〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD971:IJD_971_f1"/〉An ill-defined, slightly erythematous, indurated patch with excoriation on the right forearm〈figure xml:id="f2"〉2〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD971:IJD_971_f2"/〉A vessel showing thrombus formation with many eosinophils (× 100)
Materialart:
Digitale Medien
URL:
http://dx.doi.org/10.1046/j.1365-4362.2001.00971.x
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