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Reactive angioendotheliomatosis in an infant
V Brazzelli1, F Baldini, C Vassallo
1Department of Human and Hereditary Pathology, Institute of Dermatology and Pathology, University of Pavia, Policlinico S. Matteo IRCCS, Italy.
Insights
Reactive cutaneous angioendotheliomatosis (RCA) is a rare benign vascular condition. This report details an extremely rare infant case, highlighting its benign presentation and histopathological findings.
Area of Science:
- Dermatology
- Pediatric Pathology
- Vascular Biology
Background:
- Reactive cutaneous angioendotheliomatosis (RCA) is a rare benign vascular proliferation.
- Infantile cases of RCA are exceptionally uncommon, presenting diagnostic challenges.
Observation:
- A 3-month-old infant presented with six small, purpuric papules on the thighs and neck.
- The infant remained in good general health, with no systemic involvement or fever.
Findings:
- Histopathology revealed intravascular aggregates of large mononucleated cells within dilated vessels.
- Immunohistochemistry showed positive staining for Ulex europaeus agglutinin 1 (UEA-1), Factor VIII-RA, and CD34.
- No pericyte proliferation or inflammation was observed around the affected vessels.
Implications:
- This case expands the understanding of RCA presentation in infants.
- The benign course and characteristic histopathology aid in differentiating RCA from other pediatric vascular lesions.
- Further research into the pathogenesis of infantile RCA may be warranted.
Abstract:
Reactive cutaneous angioendotheliomatosis (RCA) is an uncommon benign disease characterized by intravascular proliferation of endothelial cells. The observation of RCA in infants is exceedingly rare. We describe a case of RCA in a 3-month-old infant. The lesions were characterized by six small purpuric papules (1-2 mm in diameter), distributed on the thighs and neck. The general condition of the patient was good, with no lymphadenopathy, systemic involvement, or fever. The histopathologic features of a papule were characterized by the presence of cohesive aggregates of large mononucleated cells protruding into the lumina of dilated vessels and filling some of them completely. Neither an inflammatory infiltrate nor a proliferation of pericytes were present around blood vessels. Intravascular proliferating cells demonstrated positive staining for Ulex europaeus agglutinin 1 (UEA-1) and for Factor VIII-RA and CD34 antigens. The course of the disease was unremarkable with persistence of the lesions for 8 months; no treatment was started.