eMedicine Specialties > Dermatology > Diseases of the Vessels

Lymphangiectasia: 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

Dermatitis Herpetiformis
Metastatic Carcinoma of the Skin
Herpes Simplex
Stewart-Treves Syndrome
Herpes Zoster
Warts, Genital
Lymphangioma
Warts, Nongenital
Malignant Melanoma

Other Problems to Be Considered

A lymphangiectatic variant of pilomatricoma has been described (del Pozo, 2004).

Workup

Other Tests

  • Immunohistochemistry
    • This study is important in differentiating lymphangiomas from hemangiomas in difficult cases.
    • Factor VIII–related antigen testing demonstrates positive results in the endothelial cells of hemangiomas but negative or weakly positive results in the endothelium of lymphangiomas.
    • Ulex europaeus I testing results are positive in the endothelial cells of lymphangiectases and hemangiomas.
    • Immunoperoxide staining with QBEnd10 (anti-CD34) antibodies shows positive reactivity results only in endothelial cells lining the blood vessels of the dermis.

Procedures

  • Skin biopsy: The diagnosis of lymphangiectasia is primarily based on clinical history and conventional light microscopy findings.

Histologic Findings

On microscopic examination, lymphangiectases are characterized by large, dilated lymph vessels lined by a single layer of endothelial cells. They are usually found in the papillary dermis and the reticular dermis. Involvement in the deeper dermis is rare. The overlying epidermis is usually associated with acanthosis and hyperkeratosis but is less marked than in angiokeratomas. At times, acquired lymphangiectases grow above the level of adjacent skin.

Lymphangiectases lack the subcutaneous muscle-coated cisternae characteristic of lymphangioma circumscriptum. Although no specific histologic criteria exist with which to differentiate lymphangioma circumscriptum from lymphangiectasia, some believe that the absence of subcutaneous cisternae in lymphangiectases is a true histologic difference and that this finding reflects the difference in the pathogenesis of both disorders.

More on Lymphangiectasia

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

References

  1. Ahmed DD, Waldorf JC, Randle HW. Cutaneous lymphangiectasis: treatment with sclerotherapy. Plast Reconstr Surg. Feb 1998;101(2):434-6. [Medline].

  2. Ambrojo P, Cogolludo EF, Aguilar A, et al. Cutaneous lymphangiectases after therapy for carcinoma of the cervix--a case with unusual clinical and histological features. Clin Exp Dermatol. Jan 1990;15(1):57-9. [Medline].

  3. Celis AV, Gaughf CN, Sangueza OP, Gourdin FW. Acquired lymphangiectasis. South Med J. Jan 1999;92(1):69-72. [Medline].

  4. Di Leonardo M, Jacoby RA. Acquired cutaneous lymphangiectasias secondary to scarring from scrofuloderma. J Am Acad Dermatol. Apr 1986;14(4):688-90. [Medline].

  5. Garcia-Doval I, de la Torre C, Losada A, et al. Acquired cutaneous lymphangiectasia in a patient with cirrhotic ascites. J Eur Acad Dermatol Venereol. Sep 1999;13(2):109-12. [Medline].

  6. Handfield-Jones SE, Prendiville WJ, Norman S. Vulval lymphangiectasia. Genitourin Med. Oct 1989;65(5):335-7. [Medline].

  7. Harwood CA, Mortimer PS. Causes and clinical manifestations of lymphatic failure. Clin Dermatol. Sep-Oct 1995;13(5):459-71. [Medline].

  8. Harwood CA, Mortimer PS. Acquired vulval lymphangiomata mimicking genital warts. Br J Dermatol. Sep 1993;129(3):334-6. [Medline].

  9. Hunt SJ, Santa Cruz DJ. Vascular tumors of the skin: a selective review. Semin Diagn Pathol. Aug 2004;21(3):166-218. [Medline].

  10. Landthaler M, Hohenleutner U, Braun-Falco O. Acquired lymphangioma of the vulva: palliative treatment by means of laser vaporization carbon dioxide. Arch Dermatol. Jul 1990;126(7):967-8. [Medline].

  11. Loche F, Schwarze HP, Bazex J. Treatment of acquired cutaneous lymphangiectasis of the thigh and vulva with a carbon dioxide laser. Acta Derm Venereol. Jul 1999;79(4):335. [Medline].

  12. Mallett RB, Curley GK, Mortimer PS. Acquired lymphangioma: report of four cases and a discussion of the pathogenesis. Br J Dermatol. Apr 1992;126(4):380-2. [Medline].

  13. Meisler DM, Eiferman RA, Ratliff NB, Burns CD. Surgical management of conjunctival lymphangiectasis by conjunctival resection. Am J Ophthalmol. Oct 2003;136(4):735-6. [Medline].

  14. Mendiratta V, Harjai B, Sardana K. Tubercular lymphadenitis with lymphangiectases of the vulva. J Eur Acad Dermatol Venereol. Mar 2005;19(2):264-5. [Medline].

  15. Moon SE, Youn JI, Lee YS. Acquired cutaneous lymphangiectasia. Br J Dermatol. Aug 1993;129(2):193-5. [Medline].

  16. Mu XC, Tran TA, Dupree M, Carlson JA. Acquired vulvar lymphangioma mimicking genital warts. A case report and review of the literature. J Cutan Pathol. Mar 1999;26(3):150-4. [Medline].

  17. Noel AA, Gloviczki P, Bender CE, et al. Treatment of symptomatic primary chylous disorders. J Vasc Surg. Nov 2001;34(5):785-91. [Medline].

  18. Peixoto CA, Figueiredo-Silva J. Fine structure of intrascrotal lymphatic vessels infected by Wuchereria bancrofti adult worms. J Submicrosc Cytol Pathol. Jan-Apr 2001;33(1-2):125-31. [Medline].

  19. Pena JM, Ford MJ. Cutaneous lymphangiectases associated with severe photoaging and topical corticosteroid application. J Cutan Pathol. Apr 1996;23(2):175-81. [Medline].

  20. Schwab RA, McCollough ML. Acquired vulvar lymphangiomas: a sequela of radiation therapy. Cutis. Mar 2001;67(3):239-40. [Medline].

  21. Sener SF, Milos S, Feldman JL, et al. The spectrum of vascular lesions in the mammary skin, including angiosarcoma, after breast conservation treatment for breast cancer. J Am Coll Surg. Jul 2001;193(1):22-8. [Medline].

  22. Ziv R, Schewach-Millet M, Trau H. Lymphangiectasia. A complication of thoracotomy for bronchial carcinoid. Int J Dermatol. Mar 1988;27(2):123. [Medline].

  23. del Pozo J, Martínez W, Yebra-Pimentel MT, Fonseca E. Lymphangiectatic variant of pilomatricoma. J Eur Acad Dermatol Venereol. Sep 2004;18(5):575-6. [Medline].

  24. el Sayed F, Bazex J, Bouissou X, et al. Acquired cutaneous lymphangiectasia mimicking plantar warts. Br J Dermatol. Jun 1995;132(6):1014-6. [Medline].

Further Reading

Keywords

acquired lymphangioma, acquired lymphangiectasia, secondary lymphangioma, lymphangioma circumscriptum

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

James Fulton Jr, MD, PhD, Medical Director, Fulton Skin Institute
James Fulton Jr, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Cosmetic Surgery, American Academy of Dermatology, Phi Beta Kappa, and Sigma Xi
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: 3M Pharmaceutical Grant/research funds Other; Graceway Pharmaceuticals Grant/research funds Other

Managing Editor

Rosalie Elenitsas, MD, Associate Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System
Rosalie Elenitsas, MD is a member of the following medical societies: American Society of Dermatopathology
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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