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Congenital and Disseminated Pyogenic Granuloma-like Vascular Lesions

Abstract : We report an exceptional case of multiple cutaneous and visceral neonatal pyogenic granuloma (PG) initially suggestive of a diffuse neonatal haemangiomatosis. CASE REPORT A full-term female newborn, with no significant past medical history, was referred to our department for treatment of an acute respiratory distress syndrome of neurological origin at day 8 of life. At birth, she presented with 3 small angiomatous papules and 4 subcutaneous nodules suggestive of neonatal hae-mangiomatosis (NH) (Fig. 1). A brain MRI revealed a highly vascularised brain stem tumour suggestive of glioma (Fig. 2), associated with 2 abnormal hepatic lesions consistent with infantile haemangiomas (IH) on ultrasound and CT scan. Methylprednisolone was started for the suspected glioma-associated oedema, and vincristine and propranolol were introduced for NH. After initial improvement, an acute intracranial hypertension related to cystic evolution of the disease necessitated surgical resection at the age of 2 months. Pathological examinations of the brain, cutaneous and subcutaneous lesions were similar, showing a vascular lobular proliferation of capillaries highly suggestive of PG. The misdiagnosis of glioma was eliminated. The GLUT-1 antigen marker was negative, ruling out the diagnosis of NH-like infantile haemangioma (Fig. 3). Lymphatic marker (D2-40) was also negative and eliminated a multifocal lymphangioendotheliomato-sis with thrombocytopaenia (MLT). Cutaneous and hepatic lesions gradually regressed. She is currently in complete remission after completing a treatment over 18 months with propranolol but a spontaneous improvement can not be excluded. DISCUSSION
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Submitted on : Thursday, November 19, 2015 - 2:39:00 PM
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Stephanie Mallet, Charlotte Rebelle, Isabelle Ligi, Didier Scavarda, Corinne Bouvier, et al.. Congenital and Disseminated Pyogenic Granuloma-like Vascular Lesions. Acta Dermato-Venereologica, Society for Publication of Acta Dermato-Venereologica, 2015, 95, pp.860-861. ⟨10.2340/00015555-2078⟩. ⟨hal-01231032⟩

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