(Last Updated On: December 14, 2018)
Authors: Claudine C. Malaca, MD, Ma. Lourdes Aragon-De Veyra

Abstract

Introduction: Lymphagioma circumscriptum (LC), also known as microcystic lymphatic malformation, is thought to arise from muscular lymphatic cisterns that failed to segment during embryonic development. Clinically, this pathology manifests as red, pink, or black scattered aggregates of small, thin-walled, translucent vesicles, giving it the classic appearance of “frog-spawn on the skin.” Some lesions exhibit overlying hyperkeratosis, resulting in a verrucous appearance. Histologic examination of superficial lesions reveals solitary or grouped cystically dilated lymph vessels located within the papillary dermis. Management remains a challenge while surgical excision is regarded as the most definitive treatment with lowest rates of recurrence, but has the highest risk of complications. Other treatments reported in literature include electrodessication, Laser, Imiquimod cream, percutaneous drainage, and sclerotherapy.

Case Summary: We are presented with a 20-year old male, with multiple skin-colored to darkly erythematous fleshy, pedunculated papules and nodules, and several hemorrhagic and clear fluid-filled vesicles on the right forearm. The patient underwent 2 surgical treatments in the past only for the lesions to recur.

The clinical presentation, biopsy, together with Dermoscopy findings of multiple white-yellowish well- circumscribed roundish areas (lacunae) surrounded by pale septa and a few containing blood, concluded a diagnosis of LC. He was started on Imiquimod 5% cream once a day every other day for a month then once daily thereafter.

Conclusion: Complications are rare and prognosis of LC is excellent as it is benign. Patient would usually come in due to cosmetic concerns and for an improved quality of life.

 

Citation

 

Keywords

lymphagioma circumscriptum, lymphatic malformation

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