Congenital and Disseminated Pyogenic Granuloma-like Vascular Lesions

Abstract

International audienceWe 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. DISCUSSIO

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

“…Mallet et al suggest the term “PG‐like congenital and disseminated angiomatosis” to designate CPG considering the relevant clinical and epidemiological differences despite similarities in their histopathological findings.…”

“…Congenital pyogenic granuloma (CPG) is a rare clinical presentation. The majority of these cases affect mucosal surfaces, and there are only nine reported cases describing skin lesions . Here, we report two cases of cutaneous CPG, including one with disseminated disease.…”

“…In our cases, no systemic involvement was seen, but as it is reported to occur in half of the cases, investigation of this kind of manifestation is justified. A review suggests that the investigation should include imaging to evaluate possible involvement of the liver, lungs, and central nervous system …”

“…The authors agreed on the inability of these methods to reach the reticular dermis layer, resulting in recurrences in half of the cases. Treatment with propranolol was ineffective in one of our cases (patient 1) as reported in almost all previous studies …”

Multifocal congenital pyogenic granuloma successfully treated with oral propranolol

“…Disseminated congenital pyogenic granuloma (DCPG) is a rare condition that can mimic multifocal IH . PG is suggested to be a hyperplastic neovascular response to an angiogenic stimulus .…”