Bianchetti, Mario

Markwalder, Regula

Pfenninger, Juerg

Simonetti, Giacomo

Tönz, Martin

RERO DOC Digital Library

SHORT REPORTSevere systemic vasculitis in a neonateGiacomo D. Simonetti & Regula Markwalder &Martin Tönz & Juerg Pfenninger & Mario G. BianchettiReceived: 3 May 2006 /Accepted: 26 June 2006 / Published online: 7 September 2006# Springer-Verlag 2006Keywords Systemic vasculitis . Neonatology .GlomerulonephritisIntroductionVasculitides are unusual in neonates with the exception oftransient vasculitides in infants of mothers affected byvasculitides mediated by proteins, which are transportedacross the placenta to the fetus [1, 4, 5]. We report here asystemic neonatal vasculitis in the son of a healthy woman.Case reportA full-term male newborn, the child of a healthy Swisswoman with normal urinalysis and negative tests forhepatitis B, hepatitis C and human immunodeficiency virus,was referred for surgical repair of esophageal atresia anddistal tracheoesophageal fistula at the age of 2 days. Threedays later, the diagnosis of anastomotic leak and infectedright-sided pneumothorax was made, which required paren-teral antimicrobial treatment with clindamycin and amoxi-cillin based on positive cultures for both viridansstreptococci and coagulase-negative staphylococci. In spiteof that the child developed a severe pleural empyema, whichwas treated with thoracotomy and decortication. Theantimicrobial medication was continued up to the age of21 days. Two days later, the condition of the childdeteriorated, as indicated by the appearance of tachypnea,progressive cyanosis, and reduced diuresis. Chest x-rayfindings disclosed intraalveolar fluid accumulation. Labora-tory values were as follows: hemoglobin, 91 g/l (referencerange, 100–150 g/l); white blood cell count, 19.8×109/l(reference range, 5.0–12.5×109/l); platelets, 39×109/l (refer-ence range, 150–300×109/l); C-reactive protein, 118 mg/l(reference range, ≤5 mg/l); creatinine, 92 μmol/l (referencerange, ≤47 μmol/l). A blood film disclosed a moderateeosinophilia of 3.9×109/l (reference range: ≤0.8×109/l).Urinalysis disclosed severe proteinuria, hematuria and redblood cell casts. Two days later, a generalized maculopapularrash was noted.Antineutrophil cytoplasmic and antinuclear autoantibod-ies, and antibodies against the Ro and La particle were notpresent in either the patient or his mother. The tentativediagnosis of systemic vasculitis was confirmed by a skinbiopsy demonstrating a characteristic leucocytoclastic vas-culitis with marked eosinophilia (Fig. 1). Immunofluores-cence studies with antisera against immunoglobulins werenot performed.Medical treatment with methylprednisolone 3 mg/kgbody weight daily was followed within 24 hour by polyuriaEur J Pediatr (2007) 166:381–382DOI 10.1007/s00431-006-0242-9Giacomo D.Simonetti: supported by Fondazione Ettore e ValeriaRossi.G. D. Simonetti : J. Pfenninger :M. G. BianchettiDepartment of Pediatrics, University of Berne,Berne, SwitzerlandR. MarkwalderDepartment of Human Pathology, University of Berne,Berne, SwitzerlandM. TönzDepartment of Pediatric Surgery, University of Berne,Berne, SwitzerlandM. G. Bianchetti (*)Ospedale San Giovanni,6500 Bellinzona, Switzerlande-mail: mario.bianchetti@pediatrician.chand normalization of plasma creatinine (36 μmol/l).Urinalysis failed to disclose pathological proteinuria andhematuria 5 days later and the treatment with methylpred-nisolone was pursued for no more than 7 days. Thesubsequent acute course was uneventful. On follow-up, 5and 12 months later, the child was well and its urinalysisnormal.DiscussionThe most frequent cases of systemic neonatal vasculitisoccur in infants of mothers affected by Behçet disease,systemic lupus erythematosus, Sjögren syndrome, undif-ferentiated connective tissue disease or microscopicpolyarteritis [1, 4]. Kawasaki disease is a further systemicvasculitis that exceptionally occurs in neonates [5]. Alsoknown as Finkelstein disease, Seidlmayer disease orinfantile Schönlein-Henoch purpura, acute hemorrhagicedema of infancy is a rather frequent self-limitingcutaneous vasculitis without any systemic involvementthat presents sometimes at birth [3]. In our patient history,symptoms and signs were not compatible with thementioned conditions.The severe eosinophilic vasculitis noted in our new-born did not recur after short-term steroid treatment. Thisfact strongly argues for a vasculitis secondary either todrugs or acute infections [2, 6]. Both streptococci andstaphylococci, the causative agents of the severe thoracicinfection of our patient, have been known or suspected tocause vasculitides. On the other side, many therapeuticagents including amoxycillin and clindamycin can inducea variety of vasculitic manifestations. We feel that the roleplayed by streptococci, staphylococci or amoxycillin inthe systemic vasculitis of our patient is impossible toanswer.In conclusion, pediatricians and neonatologists should beaware that systemic vasculitides can occur in a neonateeven if his mother is not affected.References1. Boh EE (2004) Neonatal lupus erythematosus. Clin Dermatol22:125–1282. Cuellar ML (2002) Drug-induced vasculitis. Curr Rheumatol Rep4:55–593. Cunningham BB, Caro WA, Eramo LR (1996) Neonatal acutehemorrhagic edema of childhood: case report and review of theEnglish-language literature. Pediatr Dermatol 13:39–444. Fain O, Mathieu E, Lachassinne E, Buisson P, Bodemer C,Gaudelus J, Thomas M (1995) Neonatal Behcet’s disease. Am JMed 98:310–3115. Krapf R, Zimmermann A, Stocker F (1981) Lethal vasculitis ofcoronary arteries in a neonate and two infants: possible neonatalvariant of the MLNS/IPN complex? Helv Paediatr Acta 36:589–5986. Mohan N, Kerr G (2003) Infectious etiology of vasculitis:diagnosis and management. Curr Rheumatol Rep 5:136–141Fig. 1 Skin biopsy specimen showing a blood vessel (arrows) withparietal and perivascular eosinophilic and neutrophilic infiltrates.Nuclei of inflammatory cells are in part fragmented and pycnotic382 Eur J Pediatr (2007) 166:381–382

Severe systemic vasculitis in a neonate

http://doc.rero.ch/record/318894/files/431_2006_Article_242.pdf

Severe systemic vasculitis in a neonate

Abstract

Similar works

Full text

Available Versions

RERO DOC Digital Library