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Congenital, Self-regressing Tufted Angioma FREE

John Browning, MD; Ilona Frieden, MD; Eulalia Baselga, MD; Annette Wagner, MD; Denise Metry, MD
[+] Author Affiliations

Author Affiliations: Departments of Dermatology, Baylor College of Medicine, Houston, Tex (Drs Browning and Metry), University of California, San Francisco (Dr Frieden), Hospital de la Santa Creu I, Sant Pau, Barcelona, Spain (Dr Baselga), and Northwestern University, Evanston, Ill (Dr Wagner).


Arch Dermatol. 2006;142(6):749-751. doi:10.1001/archderm.142.6.749.
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Published online

ABSTRACT

Background  Tufted angioma (known in Japanese literature as angioblastoma of Nakagawa) is an uncommon, histologically benign, vascular tumor. Lesions typically present during infancy or early childhood and are most commonly reported to persist and/or expand over time. Congenital presentations are rare, as are reports of spontaneous regression.

Observations  We present a series of 5 histopathologically confirmed cases of congenital tufted angioma that spontaneously regressed during infancy or early childhood. We also review the literature, focusing on both congenital and early-onset cases in infants.

Conclusion  We recommend that observation for potential regression be considered for otherwise uncomplicated congenital or early infantile cases of tufted angioma.

Figures in this Article

Tufted angioma (TA), also known in the Japanese literature as angioblastoma of Nakagawa, is an uncommon, histologically benign, vascular tumor that most often manifests during infancy or early childhood. It shows characteristic histopathologic findings, with lobules or tufts of capillaries in the dermis. Within the tufts are benign spindle cells, which appear to push against the adjacent vessels, giving them a slitlike appearance. On low-power microscopy, the tufts have the appearance of cannonballs in the dermis.

Most cases of TA are sporadic, although familial cases have been rarely reported.1,2 There is no sex predominance, and the clinical presentation is variable. Lesions most often present as solitary tumors or large, infiltrated plaques that are dusky red or violaceous in color. Other characteristic features include increased lanugo hair, an overlying port-wine–like stain, nodularity, or cobblestoning. Tufted angiomas are most commonly reported to persist, often slowly enlarging over years, and may be tender. Kasabach-Merritt phenomenon (KMP) seems to be a rare complication of TA, more common to the kaposiform hemangioendothelioma, although these 2 lesions are considered by many to be within the same spectrum.

Congenital cases of TA are unusual, as are reports of spontaneous regression. We present a series of 5 histopathologically confirmed cases of congenital TA, 4 of which completely regressed clinically during infancy and 1 that had partially regressed clinically by the time the child was 4 years old. We also review 10 prior reports of congenital or early-onset TA.

REPORT OF CASES

In Table 1 we present 5 new cases of congenital TA that spontaneously regressed, 4 during infancy and 1 by the time the child was 4 years old. Biopsy specimens were taken from all patients, and histopathologic findings from all specimens showed classic features of TA. Complete regression of the TA during infancy was observed in 4 patients, 3 of whom had residual skin changes. One patient exhibited partial regression by age 4 years. None of our cases was complicated by KMP. One patient received an intralesional corticosteroid injection whereas the other 4 were observed without intervention.

Ten reports of congenital or early-onset TA, all confirmed by histologic results, have been previously published in the literature37; 4 of the lesions were congenital, 5 developed within the first 3 months of life, and 1 was noted by age 9 months. Tenderness or pain was noted in all. None were complicated by KMP. Eight of the 10 patients showed complete or partial regression. The 2 lesions that did not regress were noted to be less tender over time.

COMMENT

Tufted angioma is uncommon but not rare; there are more than 200 reports in the English and Japanese literature.8 However, presentation at birth is unusual, and reports of spontaneously regressing TA even more so; there are only 4 cases confirmed by histologic results reported prior to the series described herein. Regression may be a phenomenon more common to lesions that present at birth or early infancy because there are even fewer reports of spontaneous regression in patients with later presentation. In contrast, to our knowledge there is only 1 report of a congenital TA that failed to improve over time.9 Other than early onset, we found no other clinical or histopathologic features more common to spontaneously regressing vs persistent lesions.

In the cases reported herein and in prior cases, regression occurred over months to a few years. Most cases completely regressed, whereas in 3 cases partial regression was observed, with decreased induration and tenderness. Prior to regressing, slow growth often occurs (Table 1). Three of our patients had residual skin changes following complete involution, although this finding is not described in previous reports and is common to other spontaneously regressing vascular tumors such as infantile hemangioma and rapidly involuting congenital hemangioma. One child had cutaneous atrophy with prominent vasculature following involution of the TA (Figure). Another child had a residual erythematous macule following involution. The third child had an overlying eczematous dermatitis after resolution of the TA. Although it is hypothesized that local secretion of growth factors important in angiogenesis, such as interleukin 8, play a role in the development of TA,10 the mechanism for regression is unknown.

Place holder to copy figure label and caption
Figure.

A, Tufted angioma (TA) involving the right temple at age 3 days; B, the same child at age 5 months following rapid involution of the TA with mild residual erythema, and C, at age 2 years with further resolution of the erythema, revealing cutaneous atrophy with prominent underlying vasculature.

Graphic Jump Location

The differential diagnosis of TA includes infantile hemangioma, vascular (particularly venous) malformation, infantile myofibroma (hemangiopericytoma), and malignant vascular tumors, such as infantile fibrosarcoma. The congenital presentation of a TA makes it particularly challenging to differentiate clinically from rapidly involuting congenital hemangioma.11 Comparative clinical and histopathologic features of TAs and rapidly involuting congenital hemangiomas are listed in Table 2. Biopsy of tissue specimens is often necessary for definitive diagnosis of TA.

Table Graphic Jump LocationTable 2. Comparision of Tufted Angioma and Rapidly Involuting Congenital Hemangioma

Potential indications for treatment of TA, similar to those for other benign vascular tumors, include vital organ or functional compromise, significant symptoms, or need to improve appearance. We did not observe KMP in any of our cases or those reviewed; it seems to be a rare complication. Surgical excision is generally the treatment of choice for TA. For those lesions not amenable to surgery, particularly when complicated by KMP, medical therapy (generally systemic corticosteroids or vincristine sulfate) may be used.4

In conclusion, spontaneous regression of TA may occur and seems to be a phenomenon possibly more common to congenital or early infantile presentations. Whether such lesions represent the same entity as acquired, persistent cases or is a variant is unknown. We suggest that physicians consider observation for otherwise uncomplicated congenital or early infantile cases of TA before considering intervention, particularly surgical intervention.

ARTICLE INFORMATION

Correspondence: Denise Metry, MD, Department of Dermatology, One Baylor Plaza, Houston, TX 77030 (dwmetry@texaschildrenshospital.org).

Financial Disclosure: None.

Author Contributions:Study concept and design: Browning and Metry. Acquisition of data: Browning, Frieden, Baselga, Wagner, and Metry. Analysis and interpretation of data: Browning, Frieden, Wagner, and Metry. Drafting of the manuscript: Browning and Metry. Critical revision of the manuscript for important intellectual content: Browning, Frieden, Baselga, Wagner, and Metry. Study supervision: Browning, Wagner, and Metry.

REFERENCES

Heagerty  AHRubin  ARobinson  TW Familial tufted angioma. Clin Exp Dermatol 1992;17344- 345
PubMed Link to Article
Tille  J-CMorris  MABrundler  M-A  et al.  Familial predisposition to tufted angioma: identification of blood and lymphatic vascular components. Clin Genet 2003;63393- 399
PubMed Link to Article
Wong  S-NTay  Y-K Tufted angioma: a report of five cases. Pediatr Dermatol 2002;19388- 393
PubMed Link to Article
Herron  MDCoffin  CMVanderhooft  SL Tufted angiomas: variability of the clinical morphology. Pediatr Dermatol 2002;19394- 401
PubMed Link to Article
McKenna  KEMcCusker  G Spontaneous regression of a tufted angioma. Clin Exp Dermatol 2000;25656- 658
PubMed Link to Article
Jang  KAChoi  JHSung  KJ  et al.  Congenital linear tufted angioma with spontaneous regression. Br J Dermatol 1998;138912- 914
PubMed Link to Article
Lam  WYLai  FMMLook  CN  et al.  Tufted angioma with complete regression. J Cutan Pathol 1994;21461- 466
PubMed Link to Article
Ban  MKamiya  HKitajima  Y Tufted angioma of adult onset, revealing abundant eccrine glands and central regression. Dermatology 2000;20168- 70
PubMed Link to Article
Satter  EKGraham  BSGibbs  NF Congenital tufted angioma. Pediatr Dermatol 2002;19445- 447
PubMed Link to Article
Bernstein  EFKantor  GHowe  N  et al.  Tufted angioma of the thigh. J Am Acad Dermatol 1994;31307- 311
PubMed Link to Article
Berenguer  BMulliken  JBEnjolras  O  et al.  Rapidly involuting congenital hemangioma: clinical and histopathologic features. Pediatr Dev Pathol 2003;6495- 510
PubMed Link to Article
Mulliken  JBEnjolras  O Congenital hemangiomas and infantile hemangioma: missing links. J Am Acad Dermatol 2004;50875- 882
PubMed Link to Article

Figures

Place holder to copy figure label and caption
Figure.

A, Tufted angioma (TA) involving the right temple at age 3 days; B, the same child at age 5 months following rapid involution of the TA with mild residual erythema, and C, at age 2 years with further resolution of the erythema, revealing cutaneous atrophy with prominent underlying vasculature.

Graphic Jump Location

Tables

Table Graphic Jump LocationTable 2. Comparision of Tufted Angioma and Rapidly Involuting Congenital Hemangioma

References

Heagerty  AHRubin  ARobinson  TW Familial tufted angioma. Clin Exp Dermatol 1992;17344- 345
PubMed Link to Article
Tille  J-CMorris  MABrundler  M-A  et al.  Familial predisposition to tufted angioma: identification of blood and lymphatic vascular components. Clin Genet 2003;63393- 399
PubMed Link to Article
Wong  S-NTay  Y-K Tufted angioma: a report of five cases. Pediatr Dermatol 2002;19388- 393
PubMed Link to Article
Herron  MDCoffin  CMVanderhooft  SL Tufted angiomas: variability of the clinical morphology. Pediatr Dermatol 2002;19394- 401
PubMed Link to Article
McKenna  KEMcCusker  G Spontaneous regression of a tufted angioma. Clin Exp Dermatol 2000;25656- 658
PubMed Link to Article
Jang  KAChoi  JHSung  KJ  et al.  Congenital linear tufted angioma with spontaneous regression. Br J Dermatol 1998;138912- 914
PubMed Link to Article
Lam  WYLai  FMMLook  CN  et al.  Tufted angioma with complete regression. J Cutan Pathol 1994;21461- 466
PubMed Link to Article
Ban  MKamiya  HKitajima  Y Tufted angioma of adult onset, revealing abundant eccrine glands and central regression. Dermatology 2000;20168- 70
PubMed Link to Article
Satter  EKGraham  BSGibbs  NF Congenital tufted angioma. Pediatr Dermatol 2002;19445- 447
PubMed Link to Article
Bernstein  EFKantor  GHowe  N  et al.  Tufted angioma of the thigh. J Am Acad Dermatol 1994;31307- 311
PubMed Link to Article
Berenguer  BMulliken  JBEnjolras  O  et al.  Rapidly involuting congenital hemangioma: clinical and histopathologic features. Pediatr Dev Pathol 2003;6495- 510
PubMed Link to Article
Mulliken  JBEnjolras  O Congenital hemangiomas and infantile hemangioma: missing links. J Am Acad Dermatol 2004;50875- 882
PubMed Link to Article

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