AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Background

Tufted angiomas are rare vascular tumors most commonly localized to the skin and subcutaneous tissues and characterized by slow angiomatous proliferation. Although adult-onset cases have been described, tufted angiomas occur far more frequently in children. These tumors have a benign course, with no reported cases of aggressive behavior or metastases. However, tufted angiomas seldom self-involute.¹

Frequency

United States

The incidence is low. As of the year 2000, only 54 cases are described in the English-language literature in the United States and Europe.

International

The international incidence of tufted angioma is also low. However, after Nakagawa identified the condition, angioblastoma, in 1949, more cases have been described in Japan. By 2000, 157 cases had been reported.^{2, 3, 4}

Mortality/Morbidity

Tufted angioma is a benign entity. No deaths directly or indirectly attributable to complications have been reported. However, pain and tenderness are common associated symptoms, and hyperhidrosis is a frequent finding that occurs in 30% of patients.⁵

The growth pattern of tufted angioma tends to be slow and progressive (ie, occurring over 5 mo to 10 y). However, an occasional tufted angioma undergoes a rapid expansion phase that leads to localized swelling and a decreased range of motion of the structures in the affected area. One report described a patient with a painful tufted angioma on the lateral neck and jaw. The tumor also had deep extension into the underlying fascia and muscle, a rare finding.

Kasabach-Merritt syndrome (KMS), also known as platelet-trapping syndrome (PTS), occurs in association with tufted angioma in some patients. Fortunately, most of these patients have a favorable response to the treatment of PTS with aspirin and/or ticlopidine, oral betamethasone, or vincristine, without further sequelae.

Race

No racial predilection is recognized.

Sex

The prevalence of tufted angioma is equal in both sexes.

Age

Most series report that 60-70% of tufted angiomas develop before the patient is aged 5 years, and 25% of tumors appear by the time the patient is aged 1 year. The Japanese-language literature, however, reports that more than 50% of tufted angiomas appear in the patient's first year of life. Of these patients, 15% are believed to have had a blemish in the area where the tufted angioma later developed; this observation suggests a congenital mode of presentation.

Fewer than 10% of tufted angiomas develop in individuals older than 50 years, and tumors in persons older than 60-80 years are rare.^{4, 1} One 81-year-old patient with a tufted angioma of 2 years' duration is reported in the French-language literature, and a case has been described in an 84-year-old man in Japan.^{6, 7}

CLINICAL

Section 3 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

History

• Most tufted angiomas develop by the time the patient is aged 5 years, although a number of juvenile and adult cases have been reported.
• A congenital linear tufted angioma also is reported.⁸ It is localized to the skin, with no documented systemic or metastatic involvement.
• Most reported tumors occur on the upper trunk, neck, and shoulders, and fewer tumors occur on the face, scalp, and proximal extremities.
• Involvement of the feet and oral mucosa is observed, but only in rare cases.^{9, 10} In the former, the tufted angioma was located intravenously.
• A tufted angioma is generally described as a dull red, brown-red, or purple cutaneous patch or plaque.
• The patch or plaque slowly enlarges for 5 months to 10 years, after which no further growth occurs.
• Generally, the lesion is described as solitary. However, multifocal presentations are reported, although these lesions are always localized to the skin and rarely involve the subcutaneous tissues.
• Partial regression is reported, but complete regression is rare.^{4, 11, 12}
• Many tufted angiomas have associated painful episodes.
• No history of trauma preceding the appearance of the tufted angioma is reported, although 1 case report claims that a tufted angioma developed at the site of an arthropod bite.

Physical

• A tufted angioma appears as a purplish red to red-brown patch or plaque that predominantly appears on the upper thorax; neck; shoulders; and, less commonly, on the face, scalp, and proximal extremities.
• The diameter of the patch of plaque generally ranges from less than 1 cm to several centimeters.
• The lesion may be solitary or multifocal, and it can be round to polycyclic in more extensive cases (see Media File 1).
• On palpation, the lesions often have a rubbery consistency, and they may be painful.
• Ulceration is rare; this finding may be predicted from its slow rate of growth.
• Hyperhidrosis of the skin overlying the area of the tufted angioma is reported. Histologically, hyperhidrosis corresponds with areas of abundant eccrine glands.⁴
• Peripheral extension occurs, and lesions of tufted angioma are described as having a thick indurated border and a central depression, with an appearance similar to that of a doughnut.⁵
• Lesions may also appear as deep red or purple patches with scattered round-to-oval dark-red nodules on their surface.
• Occasionally, tufted angiomas may be associated with the more serious PTS or KMS. In this setting, the presence of petechiae and ecchymotic patches should alert the physician to the development of these entities.¹³

Causes

• No causes of tufted angioma have been established. Trauma does not appear to be a predisposing factor, although a report describes the appearance of a lesion of tufted angioma at the site of a previous arthropod bite.
• Several authors have noted the development of tufted angioma within port-wine stains or in the context of these stains.^{14, 15, 16}
• Some authors have postulated that high hormonal levels during pregnancy and puberty may induce the development of tufted angiomas.^{17, 18}

DIFFERENTIALS

Section 4 of 11  

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• Clinical
• Differentials
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• Follow-up
• Miscellaneous
• Multimedia
• References

Other Problems to be Considered

Kaposiform hemangioendothelioma
Juvenile capillary, or strawberry, angioma
Hemangiopericytoma
Lobular capillary hemangioma, or pyogenic granuloma
Infantile hemangioendothelioma of the parotid gland
Dabska malignant endovascular papillary angioendothelioma

WORKUP

Section 5 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Lab Studies

• No specific laboratory study is useful in the diagnosis or treatment of tufted angioma.
• Urinary levels of basic fibroblast growth factor are of no value in the diagnosis of this condition, unlike juvenile capillary angioma.
• If the coexistence of KMS is suspected, a CBC count with a determination of the platelet count, prothrombin time and/or activated partial thromboplastin time, and full disseminated intravascular coagulation profile is indicated.

Imaging Studies

• MRI studies have been proven useful in the examination of patients with deep and/or extensive tufted angiomas.
• MRI has been successful in evaluating the depth of invasion and extent of growth of tufted angiomas that extend deep into the muscle and fascia.⁵

Other Tests

• No other tests are recommended.

Procedures

• Other than biopsy for diagnosis and the surgical treatment described below, no other procedures are indicated.

Histologic Findings

Tufted angiomas have a specific histologic pattern. They are characterized by the lobular arrangement of densely cellular capillaries, which appear similar to cannonballs, that are distributed throughout the dermis.¹⁹ The vascular tufts consist of tightly packed hypertrophic endothelial cells with scanty cytoplasm and nuclei that are round, ovoid, or fusiform.

Mitoses are abundant, but atypia and pleomorphism are rare. Occasionally, hemosiderin deposits may be observed within the endothelial cells. Capillary spaces are narrow and elongated, and they are more noticeable in the periphery of the lobules, where they have a characteristic half-moon shape.²⁰

The capillary lobules are present throughout the dermis and subcutaneous tissue; the epidermis is uninvolved in most cases. Rare cases with fascial and muscular involvement are reported.⁵ The surrounding appendageal structures of the dermis are unaffected, although hypertrophy of neighboring eccrine sweat glands is reported. No edema or inflammation surrounds the vascular lobules.

TREATMENT

Section 6 of 11  

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• Follow-up
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Medical Care

Medical treatment is not necessary, given the benign nature and slow progression of this vascular tumor. Generally, observation alone is the recommended course of action.

Surgical Care

• Complete surgical excision, if chosen, allows permanent removal of the tumor, although local recurrence of some surgically excised tumors is observed.
• Cryosurgery, radiation therapy, and electrocoagulation are successful to varying degrees in many patients.²¹
• More recently, use of the pulsed dye laser has been satisfactory treatment of a number of patients with tufted angioma.²¹

Consultations

• Typically, consultations are not necessary because of the localized nature of this entity.
• If the tufted angioma is on the face or other area of aesthetic concern, a psychologist may be consulted.
• If KMS is suspected in a child, consultation with a hematologist and/or pediatrician may be indicated.

MEDICATION

Section 7 of 11  

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Because of the benign, slowly progressive, and occasionally self-remitting nature of tufted angioma, observation is the best course of action.

The use of no specific drug is indicated in the treatment of patients with tufted angioma. However, anecdotal cases that benefited from several agents are reported, as follows:

• Systemic steroids were successfully used in 1 patient with tufted angioma.²²
• Topical steroids decreased painful episodes in 1 patient.²³
• Nonsteroidal anti-inflammatory drugs (NSAIDs) also are useful in the treatment of painful episodes.²¹
• Interferon alfa therapy significantly improved a rapidly growing and locally invasive tufted angioma in 1 pediatric patient.⁵

FOLLOW-UP

Section 8 of 11  

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• Clinical
• Differentials
• Workup
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• Follow-up
• Miscellaneous
• Multimedia
• References

In/Out Patient Meds

• Outpatient medications may include topical steroids or oral NSAIDs for the treatment of painful episodes associated with tufted angioma.
• Systemic corticosteroids and subcutaneous interferon alfa injections have been used with success in a limited number of patients with tufted angioma, although this indication is not formally approved by the US Food and Drug Administration.

Deterrence/Prevention

• Because the cause of tufted angioma is poorly understood, no specific method of prevention or deterrence is recommended.

Complications

• KMS or PTS may complicate the course of tufted angioma.
• Suspect KMS in patients with tufted angioma in whom ecchymotic patches, thrombocytopenia, and/or blood-clotting abnormalities develop.
• Responses to a variety of appropriate treatment modalities are noted in patients with KMS and tufted angioma.^{24, 13}

Prognosis

• The prognosis is good for patients with tufted angioma.
• The progression of tufted angioma is limited.

Patient Education

• Inform the patient and/or his or her parents of the benign nature of the condition, its slowly progressive course, rare regression, and occasional association with KMS.

MISCELLANEOUS

Section 9 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical/Legal Pitfalls

• No specific medication is indicated for the treatment and/or induction of regression in tufted angioma.
• Therefore, when a patient with a symptomatic lesion (eg, painful episodes, rapid growth, invasion of fascia and muscle with limitation of mobility) is treated, the patient or surrogate must be made aware of the nature of the treatment, and appropriate informed consent must be obtained.

Special Concerns

• One must be aware of the possible development of KMS.
• If KMS develops, rapid diagnosis and treatment are necessary.

MULTIMEDIA

Section 10 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Media file 1:  This lesion is a tufted angioma in a 1-year-old girl. Present since birth, this bluish-red hemangioma-like plaque on the patient's right upper leg slowly enlarged and became tender during the month prior to presentation. The child was otherwise well. A skin biopsy specimen revealed dilated congested capillaries in the lower dermis and subcutis. Clusters of capillaries with hyperplastic endothelial cells were also scattered in the mid and upper parts of the dermis. The epidermis revealed a slight basket-weave orthokeratosis, with minimal acanthosis, and papillomatosis. The patient was treated conservatively with only observation. The lesion remained stable and did not progressed since presentation. Courtesy of National Skin Centre, Singapore.
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Media type:  Photo

REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Clinical
• Differentials
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• Treatment
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• Follow-up
• Miscellaneous
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• References

1. Jones EW, Orkin M. Tufted angioma (angioblastoma). A benign progressive angioma, not to be confused with Kaposi's sarcoma or low-grade angiosarcoma. J Am Acad Dermatol. Feb 1989;20(2 Pt 1):214-25. [Medline].
2. Nakagawa K. Case report of angioblastoma of the skin. Jpn J Dermatol. 1949;59:92-4.
3. Igarashi M, Oh-i T, Koga M. The relationship between angioblastoma (Nakagawa) and tufted angioma: report of four cases with angioblastoma and a literature-based comparison of the two conditions. J Dermatol. Aug 2000;27(8):537-42. [Medline].
4. Ban M, Kamiya H, Kitajima Y. Tufted angioma of adult onset, revealing abundant eccrine glands and central regression. Dermatology. 2000;201(1):68-70. [Medline].
5. Suarez SM, Pensler JM, Paller AS. Response of deep tufted angioma to interferon alfa. J Am Acad Dermatol. Jul 1995;33(1):124-6. [Medline].
6. Dewerdt S, Callens A, Machet L, Grangeponte MC, Vaillant L, Lorette G. [Acquired tufted angioma in an adult: failure of pulsed dye laser therapy]. Ann Dermatol Venereol. Jan 1998;125(1):47-9. [Medline].
7. Murakami M, Nitta Y, Ikeya T, Hara K. Two cases of angioblastoma (Nakagawa): A one-month baby and an 84-year-old man (in Japanese). Rinsho Derma (Tokyo). 1997;39:925-9.
8. Jang KA, Choi JH, Sung KJ, Moon KC, Koh JK. Congenital linear tufted angioma with spontaneous regression. Br J Dermatol. May 1998;138(5):912-3. [Medline].
9. Fukunaga M. Intravenous tufted angioma. APMIS. Apr 2000;108(4):287-92. [Medline].
10. Kleinegger CL, Hammond HL, Vincent SD, Finkelstein MW. Acquired tufted angioma: a unique vascular lesion not previously reported in the oral mucosa. Br J Dermatol. Apr 2000;142(4):794-9. [Medline].
11. Lam WY, Mac-Moune Lai F, Look CN, Choi PC, Allen PW. Tufted angioma with complete regression. J Cutan Pathol. Oct 1994;21(5):461-6. [Medline].
12. Miyamoto T, Mihara M, Mishima E, Hagari Y, Shimao S. Acquired tufted angioma showing spontaneous regression. Br J Dermatol. Dec 1992;127(6):645-8. [Medline].
13. Enjolras O, Wassef M, Dosquet C, Drouet L, Fortier G, Josset P, et al. [Kasabach-Merritt syndrome on a congenital tufted angioma]. Ann Dermatol Venereol. Apr 1998;125(4):257-60. [Medline].
14. Swerlick RA, Cooper PH. Pyogenic granuloma (lobular capillary hemangioma) within port-wine stains. J Am Acad Dermatol. May 1983;8(5):627-30. [Medline].
15. Alessi E, Bertani E, Sala F. Acquired tufted angioma. Am J Dermatopathol. Oct 1986;8(5):426-9. [Medline].
16. Kim TH, Choi EH, Ahn SK, Lee SH. Vascular tumors arising in port-wine stains: two cases of pyogenic granuloma and a case of acquired tufted angioma. J Dermatol. Dec 1999;26(12):813-6. [Medline].
17. Padilla RS, Orkin M, Rosai J. Acquired "tufted" angioma (progressive capillary hemangioma). A distinctive clinicopathologic entity related to lobular capillary hemangioma. Am J Dermatopathol. Aug 1987;9(4):292-300. [Medline].
18. Kim YK, Kim HJ, Lee KG. Acquired tufted angioma associated with pregnancy. Clin Exp Dermatol. Nov 1992;17(6):458-9. [Medline].
19. Jones EW. Dowling oration 1976. Malignant vascular tumours. Clin Exp Dermatol. Dec 1976;1(4):287-312. [Medline].
20. Prieto VG, Shea CR. Selected cutaneous vascular neoplasms. A review. Dermatol Clin. Jul 1999;17(3):507-20, viii. [Medline].
21. Descours H, Grézard P, Chouvet B, Labeille B. [Acquired tufted angioma in an adult]. Ann Dermatol Venereol. Jan 1998;125(1):44-6. [Medline].
22. Munn SE, Jackson JE, Jones RR. Tufted haemangioma responding to high-dose systemic steroids: a case report and review of the literature. Clin Exp Dermatol. Nov 1994;19(6):511-4. [Medline].
23. Bernstein EF, Kantor G, Howe N, Savit RM, Koblenzer PJ, Uitto J. Tufted angioma of the thigh. J Am Acad Dermatol. Aug 1994;31(2 Pt 2):307-11. [Medline].
24. Léauté-Labrèze C, Bioulac-Sage P, Labbé L, Méraud JP, Taïeb A. Tufted angioma associated with platelet trapping syndrome: response to aspirin. Arch Dermatol. Sep 1997;133(9):1077-9. [Medline].
25. Kamath GH, Bhat RM, Kumar S. Tufted angioma. Int J Dermatol. Dec 2005;44(12):1045-7. [Medline].

Article Last Updated: Nov 13, 2007