Congenital Disseminated Pyogenic Granuloma: Characterization of an Aggressive Multisystemic Disorder.

OBJECTIVE: To describe the clinical, radiologic, and histopathologic features of "congenital disseminated pyogenic granuloma" involving various organs with high morbidity related to cerebral hemorrhagic involvement. STUDY DESIGN: We searched the database of the Vascular Anomalies Center at Boston Children's Hospital from 1999 to 2019 for patients diagnosed as having multiple vascular lesions, visceral vascular tumors, congenital hemangiomatosis, multiple pyogenic granulomas, or multiple vascular lesions without a definite diagnosis. A retrospective review of the medical records, photographs, histopathologic, and imaging studies was performed. Only patients with imaging studies and histopathologic diagnosis of pyogenic granuloma were included. RESULTS: Eight children (5 male, 3 female) had congenital multifocal cutaneous vascular tumors. Lesions also were found in the brain (n = 7), liver (n = 4), spleen (n = 3), muscles (n = 4), bone (n = 3), retroperitoneum (n = 3), and intestine/mesentery (n = 2). Less commonly affected were the spinal cord, lungs, kidneys, pancreas, and adrenal gland (n = 1 each). The mean follow-up period was 21.8 months. The cerebral and visceral lesions were hemorrhagic with severe neurologic sequelae. The histopathologic diagnosis was pyogenic granuloma with prominent areas of hemorrhage and necrosis. The endothelial cells had enlarged nuclei, pale cytoplasm and were immunopositive for CD31 and negative for D2-40 and glucose transporter 1. CONCLUSIONS: Congenital disseminated pyogenic granuloma is a distinct multisystemic aggressive disorder that primarily affects the skin, brain, visceral organs, and musculoskeletal system. Differentiation of this entity from other multiple cutaneous vascular lesions is critical because of possible cerebral hemorrhagic involvement.

Incidence of reactive hyperplastic lesions in the oral cavity: a 10 year retrospective study in Santa Catarina, Brazil / Incidência de lesões hiperplásicas reativas na cavidade bucal: estudo retrospectivo de 10 anos em Santa Catarina, Brasil

Abstract Introduction Reactive hyperplastic lesions develop in response to a chronic injury simulating an exuberant tissue repair response. They represent some of the most common oral lesions including inflammatory fibrous hyperplasia, oral pyogenic granuloma, giant cell fibroma, peripheral ossifying fibroma, and peripheral giant cell lesions. Objective The incidence of those lesions was investigated in an oral pathology service, and the clinical characteristics, associated etiological factors, concordance between the clinical and histopathological diagnostic was determined. Methods A total of 2400 patient records were screened from 2006 to 2016. Clinical features were recorded from biopsy reports and patients' files. Results A total of 534 cases of reactive hyperplastic lesions were retrieved and retrospectively studied, representing 22.25% of all diagnoses. The most frequent lesion was inflammatory fibrous hyperplasia (72.09%), followed by oral pyogenic granuloma (11.79%), giant cell fibroma (7.30%), peripheral ossifying fibroma (5.24%), and peripheral giant cell lesions (3.55%). Females were predominantly affected (74.19%), the gingiva and alveolar ridge were the predominant anatomical site (32.89%), and chronic traumatism was presented as the main etiological factor. The age widely ranges from the 1st decade of life to the 7th. Clinically, the reactive hyperplastic lesions consisted of small lesions (0.5-2 cm) and shared a strong likeness in color to the oral mucosa. The concordance between the clinical and histopathological diagnostic was high (82.5%). Conclusion Reactive hyperplastic lesions had a high incidence among oral pathologies. The understanding of their clinical features helps to achieve a clearer clinical and etiological diagnosis, and the knowledge of factors related to their development. This may contribute to adequate treatment and positive prognosis.

Resumo Introdução As lesões hiperplásicas reativas se desenvolvem em resposta a uma lesão crônica que estimula uma resposta acentuada de reparo tecidual. Elas representam uma das lesões orais mais comuns, inclusive hiperplasia fibrosa inflamatória, granuloma piogênico oral, fibroma de células gigantes, fibroma periférico ossificante e lesão periférica de células gigantes. Objetivo A incidência dessas lesões foi investigada em um serviço de patologia bucal e as características clínicas, os fatores etiológicos associados e a concordância entre os diagnósticos clínico e histopatológico foram determinados. Método Foram selecionados 2.400 registros de pacientes entre 2006 e 2016. As características clínicas foram registradas a partir de laudos de biópsia e dos prontuários dos pacientes. Resultados Um total de 534 casos de lesões hiperplásicas reativas foram recuperados e retrospectivamente estudados, representando 22,25% de todos os diagnósticos. A lesão mais frequente foi hiperplasia fibrosa inflamatória (72,09%), seguida por granuloma piogênico oral (11,79%), fibroma de células gigantes, (7,30%), fibroma periférico ossificante (5,24%) e lesão periférica de células gigantes (3,55%). O sexo feminino foi predominante (74,19%), a gengiva e a crista alveolar foram o local anatômico predominante (32,89%) e o traumatismo crônico foi demonstrado como o principal fator etiológico. A idade variou desde a 1ª década de vida até a 7ª. Clinicamente, as LHR consistiram em pequenas lesões (0,5 a 2 cm) que apresentaram uma forte semelhança de cor com a mucosa oral. A concordância entre o diagnóstico clínico e histopatológico foi alta (82,5%). Conclusão As lesões hiperplásicas reativas apresentaram alta incidência entre as patologias bucais. A compreensão das características clínicas ajuda na realização de um diagnóstico clínico e etiológico mais claro, bem como determinar os fatores relacionados ao seu desenvolvimento. Dessa forma contribui para um tratamento adequado e um prognóstico positivo.

Congenital cutaneous pyogenic granuloma: Report of two cases and review of the literature.

Congenital cutaneous pyogenic granuloma is a rare benign vascular tumor with clinical and histopathological features similar to infantile hemangioma. It usually presents as a red, pedunculated and highly friable papule. On histopathological analysis, one can see a capillary vessel proliferation with lobular pattern and endothelial proliferation. The differential diagnosis is based on negativity of glucose transporter 1 (GLUT1) immunochemistry studies. We report two infants with congenital pyogenic granuloma, one with a unique cutaneous lesion and the other with multiple lesions affecting both skin and mucosal surfaces. These two cases highlight the importance of the differential diagnosis based on the GLUT1 immunochemistry analysis considering the distinct treatments required to these infant vascular tumors.

Incidence of reactive hyperplastic lesions in the oral cavity: a 10 year retrospective study in Santa Catarina, Brazil.

INTRODUCTION: Reactive hyperplastic lesions develop in response to a chronic injury simulating an exuberant tissue repair response. They represent some of the most common oral lesions including inflammatory fibrous hyperplasia, oral pyogenic granuloma, giant cell fibroma, peripheral ossifying fibroma, and peripheral giant cell lesions. OBJECTIVE: The incidence of those lesions was investigated in an oral pathology service, and the clinical characteristics, associated etiological factors, concordance between the clinical and histopathological diagnostic was determined. METHODS: A total of 2400 patient records were screened from 2006 to 2016. Clinical features were recorded from biopsy reports and patients' files. RESULTS: A total of 534 cases of reactive hyperplastic lesions were retrieved and retrospectively studied, representing 22.25% of all diagnoses. The most frequent lesion was inflammatory fibrous hyperplasia (72.09%), followed by oral pyogenic granuloma (11.79%), giant cell fibroma (7.30%), peripheral ossifying fibroma (5.24%), and peripheral giant cell lesions (3.55%). Females were predominantly affected (74.19%), the gingiva and alveolar ridge were the predominant anatomical site (32.89%), and chronic traumatism was presented as the main etiological factor. The age widely ranges from the 1st decade of life to the 7th. Clinically, the reactive hyperplastic lesions consisted of small lesions (0.5-2cm) and shared a strong likeness in color to the oral mucosa. The concordance between the clinical and histopathological diagnostic was high (82.5%). CONCLUSION: Reactive hyperplastic lesions had a high incidence among oral pathologies. The understanding of their clinical features helps to achieve a clearer clinical and etiological diagnosis, and the knowledge of factors related to their development. This may contribute to adequate treatment and positive prognosis.