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"Pseudoerysipelas" in a Child with Turner's Syndrome

Identifieur interne : 005276 ( Istex/Corpus ); précédent : 005275; suivant : 005277

"Pseudoerysipelas" in a Child with Turner's Syndrome

Auteurs : Christopher Randolph ; Howard Faden

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RBID : ISTEX:B04B9D7800EE0C459E79D1D6D95F08EF56FE37C6
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DOI: 10.1177/000992288502400107

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ISTEX:B04B9D7800EE0C459E79D1D6D95F08EF56FE37C6

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<meta-value>34 "Pseudoerysipelas" in a Child with Turner's Syndrome SAGE Publications, Inc.1985DOI: 10.1177/000992288502400107 Christopher Randolph MD Department of Pediatrics, Divisions of Allergy and Infectious Diseases, State University of New York, School of Medicine, Buffalo, New York Howard Faden MD Department of Pediatrics, Divisions of Allergy and Infectious Diseases, State University of New York, School of Medicine, Buffalo, New York Received for publication February 1984, revised March 1984, and accepted May 1984. TURNER'S SYNDROME is a genetic disorder of females characterized by an abnormality of an X chromosome in association with short stature, a webbed neck, congenital peripheral lymphedema of the extremities, widely spaced nipples, cubitus vatgus, scoliosis, gonadal dysgenesis, as well as renal and cardiovascular anomalies. The lymphedema which is secondary to lymphatic obstruction tends to decrease or disappear early in life. Despite abnormalities in lymphatic drainage, infection has not been a common problem. In the present report, we describe a child with Turner's syndrome, mild persistent peripheral lymphedema, and multiple episodes of an apparent infectious process in the extremities. Case Report The patient was a 14-year-old girl with Turner's syndrome complicated by the presence of mitral valve prolapse, horseshoe kidney, and lymphedema of both hands and feet. She was admitted to the hospital with fever and painful swelling of her right hand. Her history was remarkable for similar complaints on 13 3 separate occasions over the previous 9 years. 2635 The most recent illness began with fever of 39.1 °G in association with painful swelling and erythema of her right hand on the day of admission. Her WBC count on admission was 16,600 per Al with 53% polynorphonuclear cells, 40% bands, 2% lymphocytes, and 5% monocytes. Erythrocyte sedimentation rate was 16 mm/hr (normal 1-10). 131ood, throat, and soft tissue aspirate cultures prior to antibiotic therapy were negative. Because of the pattern of illness and the response during previous hc~spitalizatic~rrs, she was treated with intravenous penicillin G, 100,000 units/ kg/day. Her symptoms improved within 24 hours and the intravenous therapy was switched to oval penicillin VK, 250 mg po qid. She was discharged 2 days later n aarkedly improved and with little residual tenderness or erythema. Comment This episode typifies the pattern of disease present in each of the preceding illnesses described in Table 1. Eleven of the other episodes required hospitlrlization for progressive pain, tenderness, and swelling. The illnesses were characterized by a rapid onset of warmth, redness, swellin~, pain, and tenderness of a single extremity. The right hand was involved in the majority of instances. The symptoms rapidly resolved after initiation of treatment with a parenteral penicillin. Symptoms were absent by 7 days of therapy. Therapy by the crrz~l route was sufficiently effective on two occasions to preclude hospitalization. The peak WBC counts ranged between 7500 to 20,800 cells/ ~I.:~ with the proportion of band forms varying from 2 to 54 percent. The erythrocyte sedimentation rate, when obtained, was elevated in till but one instance. Blood cultures were consistentty sterile. Aspirates of soft tissues were sterile, The clinical presentation in conjunction with significant laboratory changes, including (eukc~c;ytc~sis, a shift to the left, increased ESR, as well as the seemingly rapid response to antibiotics, all suggested an infectious etiology to the problem. An immunologic evaluation of the child was performed because of the frequency of the illness and the presumed infectious nature of them. In brief, quantitative immunogtobutins, including IgE, specific immunoglobulins to rubella, CMV, EBV, and complement components 3 and 4, as well as total heniolytic complement activity, were tested and found to be present in normal concentrations. Cellular studies revealed normal numbers and function of B and T lymphocvtes. isleut.rophils were present in normal, numbers; they chemotaxed and reduced nitro blue tetraxotium (NBT) dye normally, Because of the prominent role of group A streptococcus in cellulitis, we looked for streptococci in throat, blood, and soft tissue cultures obtained on several occasions but found none. An atitistreptolysin t) (ASO) titer of 664 TU was obtained following one episode. The p</meta-value>
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