Abbreviations AHEI acute haemorrhagic oedema of infancy á SHP SchoÈ nlein-Henoch purpura Information A 23-month-old girl was evaluated because of oedema and purpuric eruption on the upper trunk and the lower extremities, 15 days after an upper respiratory tract infection, treated with antibiotics (azitrocin). On the following days, erythematous macular skin lesions appeared over the auricles, hands, arms, feet and perineal and gluteal regions.Physical examination on admission revealed an otherwise healthy and afebrile child. Multiple purpuric, oedematous, target-like plaques were present mainly on the face, auricles, lower extremities ( Fig. 1) and upper trunk.The following laboratory tests were within the normal range: haemoglobin levels, platelet number, coagulation pro®le, blood urea, creatinine, liver enzymes, serum levels of IgG, IgA and IgM, C 3 , C 4 , antistreptolysin O titre, anti-nuclear and anti-DNA antibodies, and urinalysis. Stool examination and throat culture were negative. White blood cell count revealed 22,000/mm 3 leucocytes (with 76% neutrophils) and the erythrocyte sedimentation rate was elevated at 22 mm/h. Circulating immune complexes were positive.All cutaneous lesions regressed spontaneously without any speci®c treatment within 3 weeks. Up to now, after 2-year follow up there is no recurrence.
Ehlers-Danlos syndrome is a complex hereditary connective tissue disorder that is characterized by abnormalities of the skin and joints and visceral and neurological manifestations. At present, at least 11 forms are recognized on the basis of their clinical characteristics, methods of transmission, and biochemical defect. The neurologic manifestations include cerebrovascular disease, peripheral neuropathy, plexopathy, periventricular subependymal heterotopias, and epilepsy. Previously, 2 females were reported to be affected with subependimal periventricular heterotopias and Ehlers-Danlos syndrome type 1. The authors report a new case of a 12-year-old girl with similar clinical and neuroradiological features.
A considerable number of cerebral ultrasound abnormalities were observed in a cohort of infants born to mothers with autoimmune disease. However, no perinatal factors were significantly associated with this finding, suggesting the fetal brain impairment had a multi-factorial aetiology. Although no case of neuromotor delay was observed, long term neurological assessment of these babies is recommended in view of the cognitive impairment reported in previous studies.
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