Angiokeratoma Circumscriptum Clinical Presentation

  • Author: William P Baugh, MD; Chief Editor: William D James, MD   more...
 
Updated: Jan 13, 2012
 

History

  • Angiokeratoma circumscriptum lesions are most commonly found on the lower extremities as an asymptomatic solitary papule or plaque, but they can also be found in the upper extremities and the trunk.[10] One incidence of angiokeratoma circumscriptum involved an asymmetrical distribution in a systematized bandlike, segmental arrangement in the trunk, legs, and face.[11]
  • Several reports have noted angiokeratoma circumscriptum appearing on the ventral and, less commonly, the dorsal surface of the tongue.[12, 13]
  • Occasionally, multiple lesions develop, usually after adolescence.
  • Patients may present with a rapid darkening or a change of the lesion.
  • Sometimes, patients may be specifically concerned about the possibility of melanoma, given the color of the lesion.[14] See the images below. A hyperkeratotic, asymmetric, variably pigmented, A hyperkeratotic, asymmetric, variably pigmented, black 3 X 4-mm papule was found on the upper right medial part of the arm of this 18-year-old woman, who was concerned about melanoma. The histologic analysis revealed a thrombosed angiokeratoma circumscriptum. Close-up view of an asymmetric black angiokeratomaClose-up view of an asymmetric black angiokeratoma mimicking a melanoma.
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Physical

  • The primary lesions of angiokeratoma consist of elevated, warty, dark red to purple, slightly compressible papules.
  • Small nodules or plaques can also be seen.
  • Sometimes, a linear distribution (with bands or streaks) of papules develops.
  • A rough hyperkeratotic scale is often found over the surface and the edges of these papules due to epithelial hyperplasia and hyperkeratosis.
  • The lesions often have irregular borders and associated pigmentation, which is mostly attributable to intraepidermal hemorrhage or associated hemosiderin pigment deposition in the dermis.
  • If excoriated or traumatized, angiokeratomas may present with epithelial erosion and bleeding.
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Causes

The cause of angiokeratoma circumscriptum is unknown. Several causal factors, such as congenital development, pregnancy, trauma,[15] subcutaneous hematomas, and tissue asphyxia, have all been proposed (see Pathophysiology).

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Contributor Information and Disclosures
Author

William P Baugh, MD  Assistant Clinical Professor of Dermatology, University of California Irvine School of Medicine and Western School of Medicine; Medical Director, Full Spectrum Dermatology; Consulting Staff, Department of Dermatology, St Jude Medical Center

William P Baugh, MD is a member of the following medical societies: American Academy of Dermatology, American Society for Laser Medicine and Surgery, and Christian Medical & Dental Society

Disclosure: Nothing to disclose.

Coauthor(s)

Cynthia L Chen  Western University of Health Sciences College of Osteopathic Medicine of the Pacific

Cynthia L Chen is a member of the following medical societies: American Osteopathic Association, American Osteopathic College of Dermatology, California Medical Association, and Los Angeles County Medical Association

Disclosure: Nothing to disclose.

Terry L Barrett, MD  Clinical Professor of Dermatology and Pathology, University of Texas Southwestern School of Medicine; Director, ProPath Dermatopathology, Dallas, Texas

Terry L Barrett, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Medical Association, American Society of Dermatopathology, College of American Pathologists, and United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

Specialty Editor Board

Timothy McCalmont, MD  Director, UCSF Dermatopathology Service, Professor of Clinical Pathology and Dermatology, Departments of Pathology and Dermatology, University of California at San Francisco; Editor-in-Chief, Journal of Cutaneous Pathology

Timothy McCalmont, MD is a member of the following medical societies: Alpha Omega Alpha, American Medical Association, American Society of Dermatopathology, California Medical Association, College of American Pathologists, and United States and Canadian Academy of Pathology

Disclosure: Apsara Consulting fee Independent contractor

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Rosalie Elenitsas, MD  Herman Beerman 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 Academy of Dermatology and American Society of Dermatopathology

Disclosure: Lippincott Williams Wilkins Royalty Textbook editor; DLA Piper Consulting fee Consulting

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

William D James, MD  Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology

Disclosure: Elsevier Royalty Other

References

  1. Ozdemir R, Karaaslan O, Tiftikcioglu YO, Kocer U. Angiokeratoma circumscriptum. Dermatol Surg. Oct 2004;30(10):1364-6. [Medline].

  2. Feramisco JD, Fournier JB, Zedek DC, Venna SS. Eruptive angiokeratomas on the glans penis. Dermatol Online J. 2009;15(10):14. [Medline].

  3. Eskiizmir G, Gencoglan G, Temiz P, Ermertcan AT. Angiokeratoma circumscriptum of the tongue. Cutan Ocul Toxicol. Sep 2011;30(3):231-3. [Medline].

  4. Somasundaram V, Premalatha S, Rao NR, Razack EM, Zahra A. Hemangiectatic hypertrophy with angiokeratoma circumscriptum. Int J Dermatol. Jan-Feb 1988;27(1):45-6. [Medline].

  5. Sodaifi M, Aghaei S, Monabati A. Cutaneous variant of angiokeratoma corporis diffusum associated with angiokeratoma circumscriptum. Dermatol Online J. Jul 15 2004;10(1):20. [Medline].

  6. Sardana K, Koranne RV, Sharma RC, Mahajan S. Angiokeratoma circumscriptum naeviforme: rare presentation on the neck. Australas J Dermatol. Nov 2001;42(4):294-5. [Medline].

  7. Ghosh SK, Bandyopadhyay D, Ghoshal L, Haldar S. Angiokeratoma circumscriptum naeviforme: a case report of a rare disease. Dermatol Online J. Sep 15 2011;17(9):11. [Medline].

  8. Imperial R, Helwig EB. Verrucous hemangioma. A clinicopathologic study of 21 cases. Arch Dermatol. Sep 1967;96(3):247-53. [Medline].

  9. Schiller PI, Itin PH. Angiokeratomas: an update. Dermatology. 1996;193(4):275-82. [Medline].

  10. Lynch PJ, Kosanovich M. Angiokeratoma circumscriptum. Arch Dermatol. Dec 1967;96(6):665-8. [Medline].

  11. Bechara FG, Happle R, Altmeyer P, Grabbe S, Jansen T. Angiokeratoma circumscriptum arranged in a systematized band-like pattern suggesting mosaicism. J Dermatol. Jul 2006;33(7):489-91. [Medline].

  12. Yildirim M, Kilinc N, Oktay MF, Topcu I. A case of solitary angiokeratoma circumscriptum of the tongue. Kulak Burun Bogaz Ihtis Derg. 2007;17(6):333-5. [Medline].

  13. Kumar MV, Thappa DM, Shanmugam S, Ratnakar C. Angiokeratoma circumscriptum of the oral cavity. Acta Derm Venereol. Nov 1998;78(6):472. [Medline].

  14. Goldman L, Gibson SH, Richfield DF. Thrombotic angiokeratoma circumscriptum simulating melanoma. Arch Dermatol. Mar 1981;117(3):138-9. [Medline].

  15. Foucar E, Mason WV. Angiokeratoma circumscriptum following damage to underlying vasculature. Arch Dermatol. Mar 1986;122(3):245-6. [Medline].

  16. Ilyas EN, Seykora JT, Heymann WR. Acquired agminated acral angioma: a novel vascular lesion. Arch Dermatol. May 2005;141(5):646-7. [Medline].

  17. Rossi A, Bozzi M, Barra E. Verrucous hemangioma and angiokeratoma circumscriptum: clinical and histologic differential characteristics. J Dermatol Surg Oncol. Jan 1989;15(1):88-91. [Medline].

  18. Wang G, Li C, Gao T. Verrucous hemangioma. Int J Dermatol. Oct 2004;43(10):745-6. [Medline].

  19. Occella C, Bleidl D, Rampini P, Schiazza L, Rampini E. Argon laser treatment of cutaneous multiple angiokeratomas. Dermatol Surg. Feb 1995;21(2):170-2. [Medline].

  20. Pasyk KA, Argenta LC, Schelbert EB. Angiokeratoma circumscriptum: successful treatment with the argon laser. Ann Plast Surg. Feb 1988;20(2):183-90. [Medline].

  21. Gorse SJ, James W, Murison MS. Successful treatment of angiokeratoma with potassium tritanyl phosphate laser. Br J Dermatol. Mar 2004;150(3):620-2. [Medline].

  22. del Pozo J, Fonseca E. Angiokeratoma circumscriptum naeviforme: successful treatment with carbon-dioxide laser vaporization. Dermatol Surg. Feb 2005;31(2):232-6. [Medline].

  23. Dolph JL, Demuth RJ, Miller SH. Angiokeratoma circumscriptum of the index finger in a child. Plast Reconstr Surg. Feb 1981;67(2):221-3. [Medline].

 
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A hyperkeratotic, asymmetric, variably pigmented, black 3 X 4-mm papule was found on the upper right medial part of the arm of this 18-year-old woman, who was concerned about melanoma. The histologic analysis revealed a thrombosed angiokeratoma circumscriptum.
Close-up view of an asymmetric black angiokeratoma mimicking a melanoma.
Low-magnification histologic view reveals some hyperkeratosis and acanthosis with rete ridges surrounding dilated vascular channels in the papillary dermis.
This mid-power histologic view reveals dilated vessels in the papillary and upper reticular dermis. The vessels are packed with red blood cells; this finding is suggestive of vessel thrombosis.
This high-power histologic view reveals some hyperkeratosis and acanthosis with rete ridges surrounding dilated vascular channels in the papillary dermis. Dilated vessels in the papillary and upper reticular dermis are observed. The vessels are packed with red blood cells; this finding is suggestive of vessel thrombosis. A normal-appearing vascular endothelium is found. No evidence of a melanocytic lesion is present.
 
 
 
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