Angiokeratoma masquerading as lymphangioma circumscriptum

Abstract

The term angiokeratoma is derived from the Greek words angio meaning “vessel,” kerat meaning “horn,” and oma meaning “tumor,” reflecting its histologic composition of dilated vascular channels with overlying epidermal hyperkeratosis. Angiokeratomas represent benign vascular proliferations involving the superficial dermis and are often characterized by their variable morphology and clinical resemblance to other dermatoses. Because of this heterogeneity, establishing an accurate diagnosis can be challenging for clinicians. We report the case of a 49-year-old female who presented with multiple, chronic, fluid-filled papules over the vulva persisting for the past 10 years. Clinically, the lesions closely resembled lymphangioma circumscriptum (LC), leading to initial diagnostic uncertainty. The significant clinical and dermoscopic overlap between angiokeratoma and LC highlights the potential for misdiagnosis. This case emphasizes the critical role of a comprehensive diagnostic approach – integrating clinical assessment, dermoscopic evaluation, and confirmatory histopathological examination – in the evaluation of atypical vulvar lesions. Correlation of these modalities is essential not only for establishing a precise diagnosis but also for guiding appropriate management and avoiding unnecessary or invasive therapeutic interventions.