Lymphangioma circumscriptum (LC) results from the development of abnormal lymphatic vasculature and is characterized by the presence of grouped vesicles filled with clear or colored fluid. Vulvar localization is uncommon. Abnormalities of the lymphatic system, such as lymphedema and cystic hygroma, are well-known features of genetic disorders such as Noonan syndrome (NS) and Turner syndrome. We report the case of a patient with NS who presented with LC of the vulva. We also discuss the expanding spectrum of clinical anomalies associated with the presentation of NS.
Download full-text PDF |
Source |
|---|
Publication Analysis
Top Keywords
Similar Publications
Lymphangioma circumscriptum, one of the common forms of lymphangioma, can be either congenital or acquired. Various acquired conditions can lead to its causation and one of the causes might be untreated hidradenitis suppurativa. So, this report highlights the need of early interventions for hidradenitis suppurativa as lymphangioma circumscriptum can be one of its consequences later in life.
View Article and Find Full Text PDFClinical features and molecular genetics of patients with RASopathies: expanding the phenotype with rare genes and novel variants.
Eur J Pediatr
December 2024
Department of Medical Genetics, Dr. Behçet Uz Children's Hospital, Izmir, Turkey.
Unlabelled: The RASopathies are a group of disorders resulting from a germline variant in the genes encoding the Ras/mitogen-activated protein kinase pathway. These disorders include Noonan syndrome (NS), cardiofaciocutaneous syndrome (CFC), Costello syndrome (CS), Legius syndrome (LS), and neurofibromatosis type 1 (NF1), and have overlapping clinical features due to RAS/MAPK dysfunction. In this study, we aimed to describe the clinical and molecular features of patients exhibiting phenotypic manifestations consistent with RASopathies.
View Article and Find Full Text PDFLymphangioma Circumscriptum on the Buccal Mucosa: A Case Report of a Rare Entity.
Cureus
September 2024
Oral and Maxillofacial Surgery, Vinayaka Mission's Sankarachariyar Dental College, Vinayaka Mission's Research Foundation (Deemed to Be University), Salem, IND.
Hamartomatous lymphatic channel proliferation causes lymphangiomas or microcystic lymphatic malformations (MLM). They are most commonly found in the head and neck, with oral occurrences a rarity. In this case, a 34-year-old woman presented with lymphangioma circumscriptum on the buccal mucosa, a condition that typically causes asymptomatic pebbly papules.
View Article and Find Full Text PDFTreatment of Oral Lymphangiomas by Coblation.
Indian J Otolaryngol Head Neck Surg
August 2024
Department of ENT, ESIC Medical College and Hospital, Faridabad, India.
Article Synopsis
- Lymphangioma is an abnormal growth of lymphatic vessels, with tongue lymphangiomas being relatively rare and traditionally associated with high recurrence rates after treatment.* -
- This study presents two cases where coblation, a form of radiofrequency ablation, was used successfully on tongue lymphangiomas, resulting in rapid recovery and minimal pain for the patients.* -
- The findings suggest that radiofrequency ablation leads to better healing and no recurrence after one year, making it a preferred treatment option for oral lymphangiomas.*
Cutaneous metastasis is rare but may indicate an advanced internal malignancy or a recurrence of a previously treated one and is usually associated with a poor prognosis. They may also pose a diagnostic problem as the clinical manifestations are variable and non-specific, which could mimic other benign conditions. We report a case of a 48-year-old female who presented with a 4-year history of erythematous papules and vesicles on the trunk mimicking lymphangioma circumscriptum.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!