OBJECTIVE: To promote proper identification and management of this rare entity by presenting a review of the literature, a case report, and illustrations of its presentation and pathology. DATA SOURCES: Sources searched included the literature contained in the National Library of Medicine's PubMed database, using the term "lymphangioma circumscriptum" combined with "vulva." Other search terms used were "capillary lymphangioma," "lymphangiectasia," and "dermal lymphangioma." Sources also included articles predating the PubMed database that were cited by other writers. METHODS OF STUDY SELECTION: Selections were restricted to the English-language medical literature published since 1960, and the search resulted in retrieval of 3272 published papers about lymphangioma. TABULATION, INTEGRATION, AND RESULTS: Congenital lymphangioma circumscriptum of the vulva has been reported in 11 patients, including one case reported and illustrated in this paper, and acquired lymphangioma circumscriptum has been reported in 20. It affects females 9-76 years old (mean 42.5 years). Clinically, it is characterized by persistent clusters of thin-walled vesicles filled with clear fluid. The diagnosis is usually made by biopsy, as these lesions often mimic such infectious diseases as molluscum contagiosum. Management options have included surgical excision of the skin and subcutaneous tissue, surface abrasion by laser or sclerosing therapy, and observation. CONCLUSION: From this comprehensive review, which includes the clinicopathologic features of both the congenital and acquired forms, illustrations from the rarest form, and a summary of treatment approaches, we conclude that lymphangioma circumscriptum poses a diagnostic challenge the risks of which are misdiagnosis and mistreatment. These risks would likely be reduced were a database of cases accessible that permitted long-term follow-up and better assessment of presenting characteristics and treatment options.
OBJECTIVE: To promote proper identification and management of this rare entity by presenting a review of the literature, a case report, and illustrations of its presentation and pathology. DATA SOURCES: Sources searched included the literature contained in the National Library of Medicine's PubMed database, using the term "lymphangioma circumscriptum" combined with "vulva." Other search terms used were "capillary lymphangioma," "lymphangiectasia," and "dermal lymphangioma." Sources also included articles predating the PubMed database that were cited by other writers. METHODS OF STUDY SELECTION: Selections were restricted to the English-language medical literature published since 1960, and the search resulted in retrieval of 3272 published papers about lymphangioma. TABULATION, INTEGRATION, AND RESULTS:Congenital lymphangioma circumscriptum of the vulva has been reported in 11 patients, including one case reported and illustrated in this paper, and acquired lymphangioma circumscriptum has been reported in 20. It affects females 9-76 years old (mean 42.5 years). Clinically, it is characterized by persistent clusters of thin-walled vesicles filled with clear fluid. The diagnosis is usually made by biopsy, as these lesions often mimic such infectious diseases as molluscum contagiosum. Management options have included surgical excision of the skin and subcutaneous tissue, surface abrasion by laser or sclerosing therapy, and observation. CONCLUSION: From this comprehensive review, which includes the clinicopathologic features of both the congenital and acquired forms, illustrations from the rarest form, and a summary of treatment approaches, we conclude that lymphangioma circumscriptum poses a diagnostic challenge the risks of which are misdiagnosis and mistreatment. These risks would likely be reduced were a database of cases accessible that permitted long-term follow-up and better assessment of presenting characteristics and treatment options.