Histologic Findings

Microscopically, the vesicles in lymphangioma circumscriptum are greatly dilated lymph channels that cause the papillary dermis to expand. They may be associated with acanthosis and hyperkeratosis. These channels are numerous in the upper dermis and often extend to the subcutis. These deeper vessels seem to have a large caliber, and they often have a thick wall that contains smooth muscle. The lumen is filled with lymphatic fluid, but it often contains red blood cells, lymphocytes, macrophages, and neutrophils. These channels are lined by flat endothelial cells, which stain positive for Ulex europaeus agglutinin-I. The interstitium often has numerous lymphoid cells and shows evidence of fibroplasia.

Nodules in cavernous lymphangioma are characterized by large, irregular channels in the reticular dermis and subcutaneous tissue that are lined by a single layer of endothelial cells. An incomplete layer of smooth muscle often lines the walls of these malformed channels. The surrounding stroma consists of loose or fibrotic connective tissue with a number of inflammatory cells. These tumors often penetrate muscle.

Cystic hygroma is indistinguishable from cavernous lymphangiomas on histology.

Treatment & Management

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Media Gallery

A 44-year-old woman with plaque on her forearm since birth that clinically appeared to be typical lymphangioma circumscriptum. Histologically, however, it had features of both hemangioma and lymphangioma.
Close-up demonstrating the clinical morphology to better advantage.
A 16-year-old obese boy with large unilateral verrucous lymphangioma.

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Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Professor of Pediatrics, Professor of Medicine, Rutgers New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, New York Academy of Medicine, Royal College of Physicians of Edinburgh, Sigma Xi, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Coauthor(s)

Geover Fernandez, MD, FAAD Staff Physician, Department of Dermatology, Rutgers New Jersey Medical School

Geover Fernandez, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society for MOHS Surgery

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Military Dermatologists, Phi Beta Kappa, Texas Dermatological Society

Disclosure: Nothing to disclose.

Jeffrey J Miller, MD Associate Professor of Dermatology, Pennsylvania State University College of Medicine; Staff Dermatologist, Pennsylvania State Milton S Hershey Medical Center

Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Society for Investigative Dermatology, Association of Professors of Dermatology, North American Hair Research Society

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Carrie L Kovarik, MD Assistant Professor of Dermatology, Dermatopathology, and Infectious Diseases, University of Pennsylvania School of Medicine

Carrie L Kovarik, MD is a member of the following medical societies: Alpha Omega Alpha

Disclosure: Nothing to disclose.

Lymphangioma Workup

Histologic Findings

References

Tables

Contributor Information and Disclosures

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