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Multiple congenital vascular lesions in a newborn: a diagnostic challenge

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We present a newborn, delivered at week 38 of gestation because of pathological cardiotocographic record with an Apgar score of 1 point at 1 and 5 min of birth, requiring… Click to show full abstract

We present a newborn, delivered at week 38 of gestation because of pathological cardiotocographic record with an Apgar score of 1 point at 1 and 5 min of birth, requiring resuscitation. Skin examination revealed multiple papules and erythematous–violaceous nodules with a vascular appearance distributed on the trunk and extremities (figure 1A–C). Thrombocytopenia was present (67 200/μL) with normal coagulation. The CT scan (figure 1D,E) revealed a large right thoracic mass with an extensive cerebral bleeding, signs of hypoxaemic encephalopathy, that led to brain death at 5 days of life. Cutaneous histological study revealed a lobulated proliferation (figure 2A–D). Similar histological lesions with intratumoral haemorrhages were observed in the lung, pleura, retroperitoneum, kidney and brain (figure 2E–H). Tissue and blood molecular study was negative for genes associated with vascular abnormalities. Clinically and histologically, it was compatible with a disseminated congenital pyogenic granuloma (DCPG). DCPG is a condition proposed in 2009 by Browning et al when describing two cases of multiple vascular skin lesions at birth, friable, with subsequent development of new lesions, immunonegative for GLUT1 and histologically compatible with a pyogenic granuloma (PG). DCPG presents a distinct multisystem aggressive pattern that primarily affects the skin, brain, visceral organs and musculoskeletal system. Histologically, pyogenic granulomas are found. Serious cerebral bleeding at birth, as our patient, has been highlighted as a characteristic of DCPG. To differentiate DCPG from common PGs, the term ‘congenital and disseminated angiomatosis pyogenic granuloma type’ has been proposed under the following criteria: congenital skin and other organ angiomatous lesions with histopathological characteristics of PG. No mutations have been found in DCPG at the moment. Maria Tejedor, Inés GraciaDarder , Pere Ramon Balliu, Rafael Félix Ramos Asensio, Pilar Jarque, Ana MartinSantiago Department of Pediatrics, Division of Neonatology, Hospital Universitari Son Espases, Palma de Mallorca, Spain Dermatology, Son Espases University Hospital, Palma De Mallorca, Spain Pathology, Son Espases University Hospital, Palma de Mallorca, Spain

Keywords: mallorca spain; son espases; multiple congenital; palma mallorca; dcpg; pyogenic granuloma

Journal Title: Archives of Disease in Childhood
Year Published: 2023

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