eMedicine Specialties > Dermatology > Diseases of the Vessels

Lymphangioma: Differential Diagnoses & Workup

Author: Geover Fernandez, MD, FAAD, Staff Physician, Department of Dermatology, University of Medicine and Dentistry New Jersey, New Jersey Medical School
Coauthor(s): Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Contributor Information and Disclosures

Updated: Mar 19, 2007

Differential Diagnoses

Dabska Tumor
Malignant Melanoma
Dermatitis Herpetiformis
Metastatic Carcinoma of the Skin
Herpes Simplex
Neurofibromatosis
Herpes Zoster
Stewart-Treves Syndrome
Lipomas
Lymphangiectasia

Other Problems to Be Considered

Other cysts and soft subcutaneous masses should be considered.
Occasionally, acquired lymphangioma circumscriptum of the vulva may mimic genital warts.2

Workup

Imaging Studies

  • MRI can define the degree of involvement and the entire anatomy of the lesion.
  • MRI can help prevent unnecessary extensive, incomplete surgical resection, because of the association with a high recurrence rate.

Other Tests

  • Immunohistochemical study is useful in differentiating lymphangiomas from hemangiomas in difficult cases.
    • Test results with factor VIII–related antigen are positive for hemangiomas but negative or weakly positive in the endothelium of lymphangiomas.
    • Immunohistochemical studies for laminin show the typical multilayered basal lamina of normal blood vessels and the discontinuous basal lamina in lymphangiomas.

Procedures

  • Biopsy: The diagnosis of lymphangiomas is based mainly on the clinical history and findings from physical examination and conventional light microscopy.

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.

More on Lymphangioma

Overview: Lymphangioma
Differential Diagnoses & Workup: Lymphangioma
Treatment & Medication: Lymphangioma
Follow-up: Lymphangioma
Multimedia: Lymphangioma
References
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References

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  2. Horn LC, Kuhndel K, Pawlowitsch T, Leo C, Einenkel J. Acquired lymphangioma circumscriptum of the vulva mimicking genital warts. Eur J Obstet Gynecol Reprod Biol. Nov 1 2005;123(1):118-20. [Medline].

  3. Bikowski JB, Dumont AM. Lymphangioma circumscriptum: treatment with hypertonic saline sclerotherapy. J Am Acad Dermatol. Sep 2005;53(3):442-4. [Medline].

  4. Ahn SJ, Chang SE, Choi JH, Moon KC, Koh JK, Kim DY. A case of unresectable lymphangioma circumscriptum of the vulva successfully treated with OK-432 in childhood. J Am Acad Dermatol. Nov 2006;55(5 Suppl):S106-7. [Medline].

  5. Allen-Davis JT, Russ P, Karrer FM, Shroyer K, Ruyle S, Odom LF. Cavernous lymphangioma presenting as a vaginal discharge in a six-year-old female: a case report. J Pediatr Adolesc Gynecol. Feb 1996;9(1):31-4. [Medline].

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Further Reading

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Keywords

cutaneous lymphangioma, lymphangioma circumscriptum, cavernous lymphangioma, cystic hygroma, abnormal lymph vessel, lymph vessel

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Contributor Information and Disclosures

Author

Geover Fernandez, MD, FAAD, Staff Physician, Department of Dermatology, University of Medicine and Dentistry New Jersey, New Jersey Medical School
Geover Fernandez, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and American Society for MOHS Surgery
Disclosure: Nothing to disclose.

Coauthor(s)

Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

Carrie L Kovarik, MD, Assistant Professor, Department of Dermatology and Dermatopathology, 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.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Director Dermatology Residency Training Program, Scott and White Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey J Miller, MD, Associate Professor, Department of Dermatology, Penn State University, Milton S Hershey Medical Center
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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