| Patient Education |
|
Click here for patient education.
|
|
You are in: eMedicine Specialties >
Dermatology > BENIGN NEOPLASMS
Angiokeratoma Circumscriptum
Article Last Updated: Feb 28, 2007
AUTHOR AND EDITOR INFORMATION
Section 1 of 11  
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 is a member of the following medical societies: American Academy of Dermatology, American Society for Laser Medicine and Surgery, and Christian Medical & Dental Society
Coauthor(s):
Terry L Barrett, MD, Director, Associate Professor, Department of Dermatology, Division of Dermatopathology and Oral Pathology, Johns Hopkins University School of Medicine;
Cynthia L Chen, BA, Clinical Assistant, Full Spectrum Dermatology
Editors: Timothy McCalmont, MD, Director, UCSF Dermatopathology Service, Professor of Clinical Pathology and Dermatology, Departments of Pathology and Dermatology, University of California at San Francisco; Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center; 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; 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; William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
Author and Editor Disclosure
Synonyms and related keywords:
AC, angiokeratomata circumscripta, angiokeratoma corporis neviform, capillary malformation
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 (AC) 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 AC in 1915 as a localized lesion on a lower extremity or the trunk. In addition, a rare manifestation of AC naviforme, with appearance on the neck, has been documented. These lesions are of clinical importance because they may clinically mimic a malignant melanoma.
In many cases, the lesions are present at birth, but they may appear in childhood or adulthood. AC 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, angiokeratoma corporis diffusum, and traumatic arteriovenous fistulas. AC has also been called angiokeratoma corporis neviform and may be best classified as a type of capillary malformation.
Pathophysiology
As Imperial and Helwig discussed in 1967, angiokeratomas are not true angiomas but rather telangiectasias of preexisting vessels. The mechanism for development of AC 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 AC, 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 AC. Overall, altered hemodynamics (typically caused by trauma) appear to produce telangiectatic vessels of the papillary dermis with an overlying reactive hyperkeratosis to the epidermis.
Frequency
United States
The frequency of AC 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
AC 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
AC may be either congenital or acquired. Lesions are not uncommonly present at birth, but development in early childhood and even adulthood has been documented.
History
- AC 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.
- Occasionally, multiple lesions develop, usually after adolescence.
- Patients may present complaining of 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.
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 AC is unknown. Several causal factors, such as congenital development, pregnancy, trauma, subcutaneous hematomas, and tissue asphyxia, have all been proposed (see Pathophysiology).
Angiokeratoma Corporis Diffusum (Fabry Syndrome)
Angiokeratoma of the Scrotum
Blue Rubber Bleb Nevus Syndrome
Cherry Hemangioma
Jellyfish Stings
Malignant Melanoma
Osler-Weber-Rendu Syndrome
Other Problems to be Considered
Acquired agminated acral angioma
Capillary aneurysm
Lymphangioma circumscriptum
Verrucous hemangioma
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 AC 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.
The lesions of Fabry disease may be differentiated from other forms of angiokeratoma because lipid-containing cytoplasmic vacuoles can sometimes be detected in endothelial cells, fibroblasts, and pericytes.
Lymphangioma circumscriptum, perhaps the most similar to AC, is a clinically distinctive vascular malformation consisting of dilated lymphatic channels arrayed within the papillary dermis. Sometimes, these lymphangiectatic spaces are filled with serosanguineous lymph fluid, but hemorrhage into the spaces can render them microscopically indistinguishable from those of angiokeratomas. At present, no immunoperoxidase markers allow definitive distinction of blood vascular endothelium from lymphatic vascular endothelium.
Verrucous hemangioma is a descriptive term that is used to classify conventional hemangiomas in which associated verrucous epidermal changes are present. Although the superficial changes in a verrucous hemangioma can be identical to those of an angiokeratoma, usually a greater degree of depth and vascular proliferation are evident in the hemangioma. In short, angiokeratomas are typically confined to the papillary dermis, whereas verrucous hemangiomas involve all levels of the dermis and may extend to involve the subcutis.
Other vascular lesions can be associated with capillary dilatation. The diverse list of diseases includes entities, such as generalized essential telangiectasia, unilateral nevoid telangiectasia, angioma serpiginosa, and Osler-Weber-Rendu disease. These syndromes lack the overlying epidermal changes seen in angiokeratomas; therefore, they are not usually included in the pathologic differential diagnosis of angiokeratoma.
Medical Care
Medical care of these superficial vascular lesions is not usually required.
Surgical Care
- AC 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.
- 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.
- 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 AC 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.
Because angiokeratomas are stable benign vascular malformations, drug therapy is not applicable to the care of patients with these lesions at this time.
Prognosis
- AC lesions are asymptomatic benign vascular malformations that require no treatment. Laser ablation has proven highly effective and may offer the best cosmetic outcome.
Medical/Legal 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.
| 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. |
 |  View Full Size Image | |
Media type: Photo
|
| Media file 2:
Close-up view of an asymmetric black angiokeratoma mimicking a melanoma. |
 | View Full Size Image | |
Media type: Photo
|
| Media file 3:
Low-magnification histologic view reveals some hyperkeratosis and acanthosis with rete ridges surrounding dilated vascular channels in the papillary dermis. |
 | View Full Size Image | |
Media type: Photo
|
| 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. |
 | View Full Size Image | |
Media type: Photo
|
| 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. |
 | View Full Size Image | |
Media type: Photo
|
- 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].
- Foucar E, Mason WV. Angiokeratoma circumscriptum following damage to underlying vasculature. Arch Dermatol. Mar 1986;122(3):245-6. [Medline].
- Goldman L, Gibson SH, Richfield DF. Thrombotic angiokeratoma circumscriptum simulating melanoma. Arch Dermatol. Mar 1981;117(3):138-9. [Medline].
- 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].
- Ilyas EN, Seykora JT, Heymann WR. Acquired agminated acral angioma: a novel vascular lesion. Arch Dermatol. May 2005;141(5):646-7. [Medline].
- Imperial R, Helwig EB. Verrucous hemangioma. A clinicopathologic study of 21 cases. Arch Dermatol. Sep 1967;96(3):247-53. [Medline].
- Kumar MV, Thappa DM, Shanmugam S, Ratnakar C. Angiokeratoma circumscriptum of the oral cavity. Acta Derm Venereol. Nov 1998;78(6):472. [Medline].
- Lynch PJ, Kosanovich M. Angiokeratoma circumscriptum. Arch Dermatol. Dec 1967;96(6):665-8. [Medline].
- Occella C, Bleidl D, Rampini P, et al. Argon laser treatment of cutaneous multiple angiokeratomas. Dermatol Surg. Feb 1995;21(2):170-2. [Medline].
- Ozdemir R, Karaaslan O, Tiftikcioglu YO. Angiokeratoma circumscriptum. Dermatol Surg. Oct 2004;30(10):1364-6. [Medline].
- Pasyk KA, Argenta LC, Schelbert EB. Angiokeratoma circumscriptum: successful treatment with the argon laser. Ann Plast Surg. Feb 1988;20(2):183-90. [Medline].
- 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].
- 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].
- Schiller PI, Itin PH. Angiokeratomas: an update. Dermatology. 1996;193(4):275-82. [Medline].
- Sodaifi M, Aghaei S, Monabati A. Cutaneous variant of angiokeratoma corporis diffusum associated with angiokeratoma circumscriptum. Dermatol Online J. 2004;10(1):20. [Medline].
- Somasundaram V, Premalatha S, Rao NR, et al. Hemangiectatic hypertrophy with angiokeratoma circumscriptum. Int J Dermatol. Jan-Feb 1988;27(1):45-6. [Medline].
- Wang G, Li C, Gao T. Verrucous hemangioma. Int J Dermatol. Oct 2004;43(10):745-6. [Medline].
- del Pozo J, Fonseca E. Angiokeratoma circumscriptum naeviforme: successful treatment with carbon-dioxide laser vaporization. Dermatol Surg. Feb 2005;31(2):232-6. [Medline].
Angiokeratoma Circumscriptum excerpt Article Last Updated: Feb 28, 2007
|
