eMedicine Specialties > Dermatology > Benign Neoplasms

Angiokeratoma Circumscriptum

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
Terry L Barrett, MD, Clinical Professor of Dermatology and Pathology, University of Texas Southwestern School of Medicine; Director, ProPath Dermatopathology, Dallas, Texas; Cynthia L Chen, Western University of Health Sciences College of Osteopathic Medicine of the Pacific

Updated: Sep 25, 2009

Introduction

Background

Angiokeratomas are a group of vascular ectasias that involve the papillary dermis and may produce papillomatosis, acanthosis, and hyperkeratosis of the epidermis. Several clinical variants of angiokeratomas exist; angiokeratoma circumscriptum is one type, and the least frequent of the other types of angiokeratomas. Overall, 8 types of angiokeratomas have been described in the literature. The first reported case dates as far back as 1889 when Mibelli described what is now known as angiokeratoma Mibelli-type on the fingers and the toes. Fabry first described angiokeratoma circumscriptum in 1915 as a localized lesion on a lower extremity or the trunk. In addition, a rare manifestation of angiokeratoma circumscriptum naeviforme, with appearance on the neck, has been documented. These lesions are of clinical importance because they may clinically mimic a malignant melanoma.¹

In many cases, the lesions are present at birth, but they may appear in childhood or adulthood. Angiokeratoma circumscriptum has been reported to coexist with angiokeratoma of Fordyce (found on the scrotum) and caviar spots (angiokeratomas of the tongue). Other clinical associations include its occurrence with Cobb syndrome, Klippel-Trenaunay syndrome, nevus flammeus, cavernous hemangiomas, hemangiectatic hypertrophy,²  angiokeratoma corporis diffusum,³ and traumatic arteriovenous fistulas. Angiokeratoma circumscriptum has also been called angiokeratoma corporis naeviform and may be best classified as a type of capillary malformation.⁴

Pathophysiology

As Imperial and Helwig discussed in 1967, angiokeratomas are not true angiomas but rather telangiectasias of preexisting vessels.⁵ The mechanism for development of angiokeratoma circumscriptum is unknown. Several causal factors, such as congenital development, pregnancy, trauma, subcutaneous hematomas, and tissue asphyxia, have all been proposed.

Interestingly, lymphangioma circumscriptum, an entity that is microscopically similar to angiokeratoma circumscriptum, has been reported to occur in a setting of damaged deep lymphatic vessels. Unlike angiokeratoma of Mibelli or angiokeratoma corporis diffusum (Fabry disease), no pattern of inheritance or associated enzyme defect has been found for angiokeratoma circumscriptum. Overall, altered hemodynamics (typically caused by trauma) appear to produce telangiectatic vessels of the papillary dermis with an overlying reactive hyperkeratosis to the epidermis.⁶

Frequency

United States

The frequency of angiokeratoma circumscriptum is unknown. However, it is probably more common than what the relatively few cases in the literature indicate. Because no associated systemic morbidity occurs, most cases remain clinically innocuous and go unreported.

Mortality/Morbidity

Angiokeratoma circumscriptum is a benign vessel ectasia involving the papillary dermis. No deaths from this entity have been reported. However, because it may clinically mimic a melanoma, morbidity may arise from attempts to render treatment for a melanoma before histologic verification is given. Furthermore, because angiokeratomata are vascular lesions, recurrent bleeding can occur. Life-threatening bleeding is not a concern, probably because of the small size of the affected vascular spaces.

Race

No ethnic predilection has been observed or reported to date.

Sex

Women are affected more commonly than men, in a ratio of approximately 3:1.

Age

Angiokeratoma circumscriptum may be either congenital or acquired. Lesions are commonly present at birth, but development in early childhood and even adulthood has been documented.

Clinical

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.⁷
• One incidence of angiokeratoma circumscriptum involved an asymmetrical distribution in a systematized bandlike, segmental arrangement in the trunk, legs, and face.⁸
• Several reports have noted angiokeratoma circumscriptum appearing on the ventral and, less commonly, the dorsal surface of the tongue.^9,10
• 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.¹¹

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.

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.

Causes

The cause of angiokeratoma circumscriptum is unknown. Several causal factors, such as congenital development, pregnancy, trauma,¹² subcutaneous hematomas, and tissue asphyxia, have all been proposed (see Pathophysiology).

Differential Diagnoses

Other Problems to Be Considered

Acquired agminated acral angioma¹³
Capillary aneurysm
Lymphangioma circumscriptum
Verrucous hemangioma^14,15

Workup

Imaging Studies

• Imaging studies are not usually indicated in the evaluation of this superficial cutaneous vascular lesion. If multiple grouped angiokeratomas are found overlying the spine in a newborn or an infant, an MRI of the spine may be prudent to exclude spinal dysraphism or Cobb syndrome.

Procedures

• The laboratory evaluation that confirms the diagnosis is a biopsy. The biopsy result will eliminate melanoma from the clinical differential.

Histologic Findings

The histopathologic features of angiokeratoma circumscriptum are similar to those seen in other clinical types of angiokeratomas (eg, Mibelli type, Fordyce type, Fabry disease). The process has an exophytic profile, with numerous ectatic thin-walled vascular channels that expand the papillary dermis. Thrombosis of these vessels is common and is responsible for the clinical mimicry of melanoma. The overlying epidermis encompasses the vascular spaces, often with a collarette, and displays variable degrees of acanthosis and hyperkeratosis. The hyperkeratotic scale may be orthokeratotic and parakeratotic. The dermal connective tissue is usually not involved, but it may contain a few siderophages.

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 of the lesion described for Image 1 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.

Treatment

Medical Care

Medical care of these superficial vascular lesions is not usually required.

Surgical Care

• Angiokeratoma circumscriptum lesions are asymptomatic benign vascular malformations that require no treatment. Nevertheless, surgical treatment is often rendered for cosmesis or because of clinical concern regarding the possibility of melanoma. Either ablation (after a firm diagnosis is established) or excision of the lesions (when the diagnosis is uncertain) can be performed. Depending on the size and the location of the angiokeratoma, simple excision may be the treatment of choice. Small lesions may also be treated with diathermy, curettage, and cautery.
• Laser ablation has proven highly effective and may offer the best cosmetic outcome. Specifically, the argon laser has been reported to effectively eliminate angiokeratomas, although associated scarring and posttreatment hypopigmentation are risks.^16,17
  • One treatment approach is to initiate treatment with an erbium or carbon dioxide laser to remove the hyperkeratotic-acanthotic epidermis, followed by the use of a laser that targets hemoglobin, such as the flash pump dye, KTP, or 880-nm diode laser.^18,19
  • Alternatively, an erbium or carbon dioxide laser may be used alone, although this approach may cause significant collateral thermal damage to the dermis and, thus, significant scarring may ensue.
  • A KTP laser or 800-nm diode laser may also be used alone, although multiple procedures may be needed for adequate treatment, depending on the underlying vessel diameter and the overlying epidermal thickness. Because of its wavelength and deeper dermal penetration, the 800-nm diode laser may be most useful for blue-black angiokeratoma circumscriptum or those with thrombosed vessels.
  • The KTP laser destroys vascular targets and is relatively specific for cutaneous blood vessels; therefore, it is ideal for the treatment of cutaneous vascular lesions. It causes less purpura than other laser systems, and patients are able to return to work immediately after treatment to the face. A typical setting for a 532-nm KTP laser for trunk angiokeratomas might be a fluence of 16-20 J/cm² with a pulse duration of 30-50 milliseconds and a spot size of 4 mm.
• Other superficial ablative therapies, such as cryotherapy, may also be effectively used to treat superficial angiokeratomas. Recurrence of the lesion after surgical excision or ablation should bring into question the original diagnosis, and histopathologic examination of the lesions should be incorporated into the evaluation of the process in such an event.

Consultations

Consult a dermatologist for both diagnostic and therapeutic suggestions. Submit all biopsy specimens to a dermatopathologist.

Medication

Because angiokeratomas are stable benign vascular malformations, drug therapy is not applicable to the care of patients with these lesions at this time.

Follow-up

Prognosis

Angiokeratoma circumscriptum lesions are asymptomatic benign vascular malformations that require no treatment. Laser ablation has proven highly effective and may offer the best cosmetic outcome.

Miscellaneous

Medicolegal Pitfalls

Angiokeratomas can present clinically in a fashion that mimics melanoma. This fact serves as a reminder that microscopic examination of clinical pigmented lesions serves as the best means for precise, specific diagnosis.

Multimedia

Media file 1: 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.

Media file 2: Close-up view of an asymmetric black angiokeratoma mimicking a melanoma.

Media file 3: Low-magnification histologic view reveals some hyperkeratosis and acanthosis with rete ridges surrounding dilated vascular channels in the papillary dermis.

Media file 4: This mid-power histologic view of the lesion described for Image 1 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.

Media file 5: 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.

References

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

2. 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].

3. 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].

4. 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].

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

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

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

8. 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].

9. 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].

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

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

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

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

14. 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].

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

16. 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].

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

18. 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].

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

20. 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].

Keywords

angiokeratoma circumscriptum, AC, angiokeratomas, angiokeratomata circumscripta, angiokeratoma corporis neviform, capillary malformation

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)

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.

Cynthia L Chen, Western University of Health Sciences College of Osteopathic Medicine of the Pacific
Disclosure: Nothing to disclose.

Medical Editor

Timothy McCalmont, MD, Director, UCSF Dermatopathology Service, Professor of Clinical Pathology and Dermatology, Departments of Pathology and Dermatology, University of California at San Francisco
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

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
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.

Managing Editor

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: Nothing to disclose.

CME Editor

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 None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

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.

The authors and editors of eMedicine gratefully acknowledge the contributions of previous Chief Editor, William D. James, MD, to the development and writing of this article.

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