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Angiokeratoma Circumscriptum Clinical Presentation

  • Author: William P Baugh, MD; Chief Editor: William D James, MD  more...
 
Updated: Jul 24, 2014
 

History

See the list below:

  • Angiokeratoma circumscriptum lesions are most commonly found on the lower extremities as an asymptomatic solitary papule or plaque, but they can also be found virtually anywhere, with dominance on the legs, upper extremities, and 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 dark purple or black 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

See the list below:

  • The primary lesions of angiokeratoma consist of elevated, hyperkeratotic, dark red to purple or black, 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.
  • When compressed with a glass slide, the dark purple or black color often blanches to red.
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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, Western University of Health Sciences; 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, Christian Medical and Dental Associations

Disclosure: Nothing to disclose.

Coauthor(s)

Cynthia L Chen, DO, DO Intern, Pacific Hospital of Long Beach, California

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

Disclosure: Nothing to disclose.

Specialty Editor Board

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

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

Disclosure: Nothing to disclose.

Rosalie Elenitsas, MD Herman Beerman 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, American Medical Association, American Society of Dermatopathology, Pennsylvania Academy of Dermatology

Disclosure: Received royalty from Lippincott Williams Wilkins for textbook editor.

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, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

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, United States and Canadian Academy of Pathology

Disclosure: Received consulting fee from Apsara for independent contractor.

References
  1. Ozdemir R, Karaaslan O, Tiftikcioglu YO, Kocer U. Angiokeratoma circumscriptum. Dermatol Surg. 2004 Oct. 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. 2011 Sep. 30(3):231-3. [Medline].

  4. Somasundaram V, Premalatha S, Rao NR, Razack EM, Zahra A. Hemangiectatic hypertrophy with angiokeratoma circumscriptum. Int J Dermatol. 1988 Jan-Feb. 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. 2004 Jul 15. 10(1):20. [Medline].

  6. Sardana K, Koranne RV, Sharma RC, Mahajan S. Angiokeratoma circumscriptum naeviforme: rare presentation on the neck. Australas J Dermatol. 2001 Nov. 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. 2011 Sep 15. 17(9):11. [Medline].

  8. Imperial R, Helwig EB. Verrucous hemangioma. A clinicopathologic study of 21 cases. Arch Dermatol. 1967 Sep. 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. 1967 Dec. 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. 2006 Jul. 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. 1998 Nov. 78(6):472. [Medline].

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

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

  16. Ilyas EN, Seykora JT, Heymann WR. Acquired agminated acral angioma: a novel vascular lesion. Arch Dermatol. 2005 May. 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. 1989 Jan. 15(1):88-91. [Medline].

  18. Wang G, Li C, Gao T. Verrucous hemangioma. Int J Dermatol. 2004 Oct. 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. 1995 Feb. 21(2):170-2. [Medline].

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

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

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