0
We're unable to sign you in at this time. Please try again in a few minutes.
Retry
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
Retry
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Observation |

Clinical Spectrum of Tufted Angiomas in Childhood:  A Report of 13 Cases and a Review of the Literature FREE

Amélie Osio, MD; Sylvie Fraitag, MD; Smail Hadj-Rabia, MD; Christine Bodemer, MD, PhD; Yves de Prost, MD, PhD; Dominique Hamel-Teillac, MD
[+] Author Affiliations

Author Affiliations: Departments of Dermatology (Drs Osio, Hadj-Rabia, Bodemer, de Prost, and Hamel-Teillac) and Pathology (Dr Fraitag), Hôpital Necker–Enfants Malades, Assistance Publique–Hôpitaux de Paris, Université Paris Descartes, Paris, France.


Arch Dermatol. 2010;146(7):758-763. doi:10.1001/archdermatol.2010.135.
Text Size: A A A
Published online

ABSTRACT

Background  Tufted angioma (TA) is a rare benign vascular tumor that mostly appears during infancy or early childhood. Histologic tufts of capillaries infiltrating the whole dermis in a “cannonball” distribution pattern associated with dilated lymphatic vessels are characteristic of the disease and confirm the diagnosis. Few case series of TA have been published, and the morphologic structure and evolution of TA seem to vary.

Observations  We describe the largest series to date of childhood TA, comprising 13 cases. All children developed lesions within the first year of life; 7 cases were congenital. We found a clear male predominance (9 of 13 children). Presentation was a nascent or florid tumor, usually a dusky red to violaceous plaque, that was indurated, firm, and sometimes associated with hyperhidrosis or hypertrichosis. Locations of the lesions included limbs, abdomen, and genitalia. Five children had spontaneous regression, 5 children had Kasabach-Merritt syndrome, and 1 child had a lesion that stabilized. Two children with painful TA had chronic coagulopathy without thrombocytopenia that was controlled by ticlopidine hydrochloride and aspirin.

Conclusions  The following 3 clinical patterns could be distinguished: TA without complications, TA complicated by Kasabach-Merritt syndrome, and TA without thrombocytopenia but with chronic coagulopathy. To our knowledge, this study is the first to describe the third pattern. Because of the aggressive nature of Kasabach-Merritt syndrome, it is essential to obtain a complete blood cell count when evaluating a child with TA.

Figures in this Article

Tufted angioma (TA) is a rare vascular tumor characterized histologically by small tufts of capillaries associated with dilated lymphatic vessels in the dermis. First reported in 1949 by Nakagawa1 as angioblastoma and then described in 1971 by MacMillan and Champion2 as progressive capillary hemangioma, these vascular tumors were finally called TA by Jones3 in 1976 after 10 cases of similar lesions with characteristic histologic findings were reported. Tufted angioma may be congenital or acquired and most often manifests during infancy or early childhood, but a few cases have been described in adults. Most frequently, lesions are initially seen as solitary tumors or infiltrating plaques that are dusky red or violaceous, sometimes associated with hyperhidrosis or hypertrichosis.4,5 Evolution of the disease seems to vary considerably, including complete or partial spontaneous regression,57 persistence over the years,5,8 or Kasabach-Merritt syndrome (KMS).9,10 Because the morphologic structure and evolution vary and because TA case series are lacking in the literature, we describe herein 13 children followed up for TA at our hospital for a mean of 7.5 years.

METHODS

We reviewed all cases of histologically diagnosed TA at Hôpital Necker–Enfants Malades, Paris, France, from January 1, 1988, to December 31, 2007. Tufted angioma was histologically defined as multiple small hypercellular nodules of capillaries with small lumina distributed in a “cannonball” pattern infiltrating the reticular dermis and sometimes the superficial subcutis. These lobules were lined by a crescent-shaped vessel, and there were some dilated lymphatic vessels scattered in a dense background. The lobules contained globular, epithelioid, or spindle-shaped cells. They were often separated by slitlike spaces containing red blood cells. There were no inflammatory cells. The large scattered vessels and some (but not all) crescent-shaped vessels stained positive for podoplanin (D240 antibody), a lymphatic marker. Some spindle-shaped or epithelioid lobular cells also stained positive, providing evidence of partial lymphatic differentiation.

We excluded children who were unavailable for follow-up. Data collected included sex, age at biopsy, age at onset of TA, initial location and morphologic structure of lesions, presence or absence of KMS, results of biologic and imaging studies (when available), treatment and outcome, associated diseases, and early and late photographs. We defined spontaneous regression as the clinical absence of superficial or deep infiltration. Nevertheless, minimal skin changes such as pigmentation could persist. Kasabach-Merritt syndrome is characterized by a vascular tumor and thrombocytopenic coagulopathy (platelet count of <150 ×103/μL), often associated with other coagulation abnormalities (low fibrinogen level of <200 mg/dL, high D-dimer level of >4 μg/mL, and increased fibrin degradation products) (to convert platelet count to ×109/L, multiply by 1.0; fibrinogen to micromoles per liter, multiply by 0.0294; and D-dimer to nanomoles per liter, multiply by 5.476).11

RESULTS

Thirteen children with TA were identified during the study period (Table 1). There were 4 girls (31%) and 9 boys (69%). The mean follow-up was 7.5 years (range, 2-19 years). No family histories of a similar illness were found.

Table Graphic Jump LocationTable 1. Characteristics of 13 Patients With Tufted Angioma (TA)
PRESENTATION

At birth, nascent or florid tumors were present that were typically poorly defined infiltrating, firm, dusky red to violaceous plaques. Other characteristic features included nodularity, hyperhidrosis, or hypertrichosis. No congenital tumors had been diagnosed in utero by ultrasonography. For noncongenital TA, the clinical presentation was similar. All TAs were painless initially. Because of infiltration, the initial size was difficult to measure but varied from 4 × 1 to 11 × 10 cm in our series.

EVOLUTION

We were able to distinguish the following 3 clinical patterns: TA without complications, TA complicated by KMS, and TA without thrombocytopenia but with chronic coagulopathy.

The first clinical pattern of TA was represented by 6 children (46%), among whom no complications occurred. The outcome of these 6 TAs was favorable, with spontaneous regression in 5 children and stabilization in 1 child. The mean follow-up period after regression was 3 years (range, 1-6 years). In patient 1, TA appeared at the age of 5 months on the chest as an expanding red violaceous plaque. Examination at age 3 years revealed a 4 × 3-cm firm vascular red mass. There were no complications, and the tumor began to decrease in size after age 6 years. At age 12 years, the tumor had totally disappeared, the skin color was normal, and there was no infiltration. The child experienced only slight perspiration in hot weather. Patient 2 was seen 15 days after birth with an expanding indurated violaceous plaque and an area of increased hair growth over the thigh. Because the tumor became warm, with hyperhidrosis but without pain or biologic abnormalities, pentoxifylline was administered at age 10 years but was ineffective. At age 19 years, the tumor remained the same size, 19 × 16 cm. Patient 3 had congenital TA as a 4 × 2-cm violaceous firm tumor over her abdomen. No complications were observed, and the size of the lesion decreased. At age 2 years, complete resolution of TA occurred spontaneously. Physical examination of patient 6 revealed a 6 × 6-cm congenital indurated blue-purple plaque on the right cheek. The mass was neither warm nor painful. Results of biologic studies were normal except for an elevated D-dimer level (1.9 μg/mL). The lesion spontaneously became less tender after 6 months, with changes in discoloration, and then decreased in size. Findings on physical examination have been normal since age 3 years. Patient 7 was seen at birth with a prominent erythematous indurated plaque on the abdomen, genitalia, and right thigh. The lesion was tender on palpation, and the range of movement in the right leg was affected. The platelet count was normal. Magnetic resonance imaging showed a large infiltrating vascular mass, and a biopsy specimen confirmed the diagnosis of TA. The tumor spontaneously and rapidly became less tender and decreased in size. At age 5 years, findings on physical examination were normal except for slightly pink-stained skin with minimal cutaneous atrophy. In patient 13, a 3.5 × 1.5-cm blue nodular plaque appeared on the back at age 1 month. It gradually became less infiltrating, with residual skin changes at age 2 years.

The second most frequent clinical pattern was TA complicated by KMS, which was diagnosed in 5 children (38%). Coagulopathy developed before age 8 months in all of them. The youngest age at which KMS was diagnosed was 1 month. No KMS was diagnosed at birth. Three of 5 children with KMS had congenital TA. Clinically, all children with KMS had an expanding, indurated, tender, and painful inflammatory mass (Figure 1). Results of biologic studies revealed profound thrombocytopenia, low fibrinogen level, and high D-dimer level. Histologic examination was performed before the onset of coagulopathy in patient 9 and during the coagulopathy phase in 3 children. A biopsy specimen was obtained from patient 12 several years after the diagnosis of KMS because of pain and venous dilatation on an area of residual hyperpigmented and infiltrated skin. Kasabach-Merritt syndrome was treated by combination therapy in all children except 1, who was cured by corticosteroid therapy alone. All others received 2 to 7 types of therapy that are reported to be effective in KMS. The treatments led to favorable outcomes. A residual lesion was observed in all 5 children, including minimal skin color changes in 2, associated subcutaneous atrophy in 1, and sequelae in muscles and joints in 2. The mean follow-up period after resolution of KMS was 7 years.

Place holder to copy figure label and caption
Figure 1.

Wide tufted angioma and Kasabach-Merritt syndrome in patient 11 at age 6 months involving genitalia area (A) and the back (B).

Graphic Jump Location

Two children (15%) demonstrated the third clinical pattern (TA without thrombocytopenia but with chronic coagulopathy). Both had a vascular tumor that became inflammatory (with warmth, erythema, and edema), painful, and indurated and was associated with fluctuating coagulopathy (fibrinogen level of <100 mg/dL, D-dimer level of >4 μg/mL, and increased fibrin degradation products) and a normal platelet count. Patient 4 had a congenital abdominal infiltrating mass that was diagnosed as TA at age 1½ years. This quiescent tumor became inflammatory and painful at age 2 years, with biologic coagulopathy (D-dimer level of 4 μg/mL and fibrinogen level of 100 mg/dL) but without thrombocytopenia (Figure 2). He was treated with a combined daily regimen of ticlopidine hydrochloride and aspirin (each 10 mg/kg of body weight); induration and pain regressed, but coagulopathy persisted. After aspirin was stopped, the tumor again became inflammatory and painful, and coagulopathy worsened. Aspirin therapy was started again, in combination with ticlopidine. Between ages 2 to 8 years, combined treatment with aspirin and ticlopidine stabilized the clinical aspects of TA, but biologic coagulopathy remained. Each time treatment was withdrawn, pain and inflammation recurred. Patient 8 was seen at age 2 months with a vascular tumor on the thigh. Histologic examination was performed at age 3 months because of local inflammation, pain, and hyperhidrosis. Results of biologic studies were normal. He was treated with corticosteroids because of functional compromise, and clinical signs improved. When corticosteroids were reduced, inflammation reappeared and was associated with coagulopathy but a normal platelet count. Magnetic resonance imaging showed a large infiltrating mass encompassing the right thigh circumferentially, not extending to the bone or muscle. A combined regimen of ticlopidine and aspirin led to clinical improvement, but coagulopathy remained for 6 years with bouts of pain, especially when treatment was suspended. Sequelae in the leg muscles and joints caused impaired mobility.

Place holder to copy figure label and caption
Figure 2.

Congenital abdominal infiltrating tufted angioma in patient 4. A, In the newborn “quiescent” state. B, The same lesion at age 2 years is inflammatory and painful, with biologic coagulopathy.

Graphic Jump Location

COMMENT

Since the initial 1989 study by Jones and Orkin,8 few case series of TA have been reported, and the largest of these included 5 children.46 Our series of 13 children illustrates several clinical patterns of presentation and evolutions of this rare vascular tumor. Because of the clinical variability in TA, histologic examination is required for diagnosis. Histologic findings were well described in the series by Jones and Orkin8 as tightly packed vessels scattered at various levels in the dermis and in the superficial subcutis, generally occurring in small tufts with a cannonball distribution pattern. Typically, the tufts are encircled by an empty crescent-shaped vessel and are surrounded by a fibrous dermis. Besides these small lobules, most lesions contain scattered larger vessels within the wall and empty lumen resembling lymphatic vessels. The vascular tufts seem to be composed of epithelioid and spindle-shaped endothelial cells closely packed together and separated by clefts containing red blood cells in a kaposiform manner. Some hyalin globules can be seen as well (Figure 3).

Place holder to copy figure label and caption
Figure 3.

Typical tufted angioma. A, Tufted angioma is characterized by round small lobules scattered in the mid and deep dermis and superficial subcutaneous tissue and in a cannonball distribution (hematein-eosin–saffron [HES], original magnification ×25). B, Typical appearance of tufts in tufted angioma with closely packed capillaries and empty crescent-shaped vessels encircling them (HES, original magnification ×100). Fibrosis is obvious in the surrounding dermis. C, High magnification shows the center of a lobule (HES, original magnification ×400). Some areas closely resemble kaposiform hemangioendothelioma or Kaposi sarcoma and show spindle cells separated by slitlike lumina containing few red blood cells (star). Some hyaline globules can also been seen (arrowhead). Besides these capillary tufts, some larger vessels with a thin wall and an empty lumen can be seen (arrow). D, Staining with D240 antibody (podoplanin) shows evidence of partial lymphatic differentiation. E, Another tufted angioma shows larger and ill-defined tufts having a tendency to coalesce, also associated with anastomosed lymphatic vessels (HES, original magnification ×25).

Graphic Jump Location

Histopathologic examination with immunohistochemistry is necessary to exclude other lesions such as hemangioma of infancy, vascular malformations, infantile myofibromatosis, and congenital dermatofibrosarcoma protuberans.12 Immunohistochemistry is positive for GLUT1 and is specific in 100% of infantile hemangiomas; it never stains infantile vascular tumors such as TA.13 Distinguishing TA from kaposiform hemangioendothelioma (KHE) is challenging because of morphologic and histopathologic similarities (Table 2). The histopathologic overlap is especially dramatic between TA and superficial KHE. Some authors consider these tumors to be on the same spectrum, while others believe they are the same entity and describe superficial KHE as TA.11,1317

Table Graphic Jump LocationTable 2. Key Differences Between Tufted Angioma and Superficial Kaposiform Hemangioendothelioma

Previous studies4,5,8 of TA described various clinical morphologic structures, including brown, blue, purple, or red poorly delineated macules, as well as indurated nodules or plaques varying in size, sometimes with a mottled appearance or with focal hyperhidrosis or hypertrichosis (Table 3). In our series, male sex clearly predominated (9 of 13 patients [69%]), as in the series by Jones and Orkin.8 In that series, the upper trunk and neck were the most commonly reported sites for TA. In other series, TA was commonly found on the limbs.5,18,19 In our series, the upper trunk was affected in only 2 children, whereas the limbs were involved in 7, the abdomen or genitalia in 3, and the cheek in 1.

Table Graphic Jump LocationTable 3. Comparison of the Present Series With Previously Published Studies

Although acquired TA has been reported in adults,1822 TA most commonly appears in infancy and early childhood4,5,8,19 This was corroborated by a 2000 review of 41 cases, more than 50% of which manifested during the first year of life.18 In our pediatric series, all TAs were observed before age 5 months. Cases of congenital TA have been described,4,68,19,2326 but their frequency is uncertain. It was estimated at 10% in 1 study19 and at 22% in a review by Igarashi et al.26 In our series, TAs were congenital in 54% (7 of 13 patients), which is higher than previously reported.

Variations in the evolution of TA have been described. Most often, TA has a slow rate of growth, typically developing and extending over several months and eventually becoming stable in size.5,8 Only 1 TA in our series stabilized in size (patient 2). Spontaneous clinical regression has also been reported.47,2326 The time to regression was less than 2 years in 95% of TAs in a review of 27 spontaneous regressions.21 In our 5 cases of spontaneous regression, 3 occurred in congenital TA and 2 in newborn TA.

Tufted angioma and KHE can be complicated by KMS.9 Since the first description by Kasabach and Merritt27 in 1940, the association of a large vascular tumor and thrombocytopenia in an infant has been considered a rare complication of vascular lesions in infancy. This thrombocytopenic coagulopathy is not observed in the more common hemangioma of infancy but occurs in uncommon vascular tumors such as TA and KHE.10 The frequency of KMS in TA is uncertain. In a study11 of 41 patients with KMS, 8 skin biopsy specimens from 26 patients revealed TA. Kasabach-Merritt syndrome was diagnosed at birth or before age 5 months in 32 of 41 cases (78%) in this study. In our 13 children, 5 (38%) were initially seen with KMS. This frequency seems higher than that in the literature, which contains a few isolated case reports but no case series.11,24,28,29 Therefore, in patients with TA, it may be advisable to obtain at least a complete blood cell count, and a platelet count of less than 150 ×103/μL should prompt a more extensive evaluation for coagulopathy.

Regarding the evolution of congenital TA, it has been suggested that spontaneous regression is more common in congenital or early-onset TA.6 Nevertheless, several studies4,7,21,2326 of congenital TA have described various courses of evolution (spontaneous regression, stabilization, or KMS). In our series, evolution was similar in congenital and noncongenital TA, including 3 cases of spontaneous regression in each group, 3 cases of KMS in the congenital group and 2 in the noncongenital group, and 1 case of coagulopathy in each group. Our study describes another clinical pattern of TA evolution, chronic coagulopathy, which we observed in 2 children. This pattern is characterized by clinical flares of TA (infiltrating, inflammatory, and painful mass) associated with coagulopathy (defined by low fibrinogen level, high D-dimer level, and increased fibrin degradation products) and a normal platelet count. Despite clinical improvement in the tumor, low-grade chronic consumptive coagulation persisted. Aspirin and ticlopidine (the treatment for KMS) were used for pain coagulopathy.10,30 This regimen led to clinical improvement in these 2 children, despite fluctuating coagulopathy for several years. Bouts of pain with inflammatory signs recurred each time treatment was withdrawn.

The management of TA is difficult, and treatment guidelines have yet to be established. Treatment may be categorized as that administered for aesthetic reasons or as that instituted because of complications or anticipated complications such as KMS or functional compromise. Several treatment regimens for TA with or without KMS have been reported, including compression therapy, surgery, laser, topical or systemic corticosteroids, interferon, and chemotherapy.4,10,30 A wait-and-see policy seems appropriate because of the benign nature of this tumor and the possibility of spontaneous regression. However, careful follow-up should be implemented, especially in early childhood, to detect complications such as KMS. No malignant changes have been described to our knowledge.

In conclusion, we describe the largest series to date of TA in childhood, with a long follow-up period. Although TA is histologically a benign vascular tumor, its outcomes range from spontaneous regression to KMS with vital or functional compromise. In our series, spontaneous regression was observed more frequently than stabilization of size, which contrasts with previous findings. Moreover, KMS was a common complication of TA in our series. Therefore, it may be advisable in patients with TA to obtain at least a complete blood cell count, and a platelet count of less than 150 ×103/μL should prompt a more extensive evaluation for coagulopathy. We also describe a new clinical pattern of evolution, chronic coagulopathy, which requires prolonged treatment. Tufted angioma should be closely monitored, especially in early childhood, when KMS is most likely to occur. In uncomplicated cases, “active nonintervention” with close surveillance is a reasonable approach, as spontaneous regression can occur.

ARTICLE INFORMATION

Correspondence: Dominique Hamel-Teillac, MD, Department of Dermatology, Hôpital Necker–Enfants Malades, Assistance Publique–Hôpitaux de Paris, Université Paris Descartes, 149 Rue de Sevres, 75743 Paris CEDEX 15, France (dominique.hamel-teillac@nck.aphp.fr).

Accepted for Publication: November 23, 2009.

Author Contributions: Dr Osio had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. Study concept and design: Osio and Fraitag. Acquisition of data: Osio, Fraitag, Bodemer, and Hamel-Teillac. Analysis and interpretation of data: Osio, Hadj-Rabia, Bodemer, de Prost, and Hamel-Teillac. Drafting of the manuscript: Osio and Hamel-Teillac. Critical revision of the manuscript for important intellectual content: Fraitag, Hadj-Rabia, Bodemer, de Prost, and Hamel-Teillac. Study supervision: Fraitag, Hadj-Rabia, Bodemer, de Prost, and Hamel-Teillac.

Financial Disclosure: None reported.

Additional Contributions: Didier Carnet, PhD, assisted with editing.

REFERENCES

Nakagawa  K Case report of angioblastoma of the skin [in Japanese]. Jpn J Dermatol 1949;5992- 94
MacMillan  AChampion  RH Progressive capillary haemangioma. Br J Dermatol 1971;85 (5) 492- 493
PubMed
Jones  EW Dowling Oration 1976: malignant vascular tumours. Clin Exp Dermatol 1976;1 (4) 287- 312
PubMed Link to Article
Herron  MDCoffin  CMVanderhooft  SL Tufted angiomas: variability of the clinical morphology. Pediatr Dermatol 2002;19 (5) 394- 401
PubMed Link to Article
Wong  SNTay  YK Tufted angioma: a report of five cases. Pediatr Dermatol 2002;19 (5) 388- 393
PubMed Link to Article
Browning  JFrieden  IBaselga  EWagner  AMetry  D Congenital, self-regressing tufted angioma. Arch Dermatol 2006;142 (6) 749- 751
PubMed Link to Article
Jang  KAChoi  JHSung  KJMoon  KCKoh  JK Congenital linear tufted angioma with spontaneous regression. Br J Dermatol 1998;138 (5) 912- 913
PubMed Link to Article
Jones  EWOrkin  M Tufted angioma (angioblastoma): a benign progressive angioma, not to be confused with Kaposi's sarcoma or low-grade angiosarcoma. J Am Acad Dermatol 1989;20 (2, pt 1) 214- 225
PubMed Link to Article
Teillac-Hamel  DAndry  PBodemer  C  et al.  Kasabach-Merritt syndrome in children [in French] Ann Pediatr (Paris) 1992;39 (7) 435- 441
PubMed
Enjolras  OWassef  MMazoyer  E  et al.  Infants with Kasabach-Merritt syndrome do not have “true” hemangiomas. J Pediatr 1997;130 (4) 631- 640
PubMed Link to Article
Enjolras  OMulliken  JBWassef  M  et al.  Residual lesions after Kasabach-Merritt phenomenon in 41 patients. J Am Acad Dermatol 2000;42 (2, pt 1) 225- 235
PubMed Link to Article
Weiss  SWGoldblum  JR Enzinger and Weiss' Soft Tissue Tumors. 5th New York, NY Elsevier Inc2008;
Enjolras  OPicard  ASoupre  V Congenital haemangiomas and other rare infantile vascular tumours [in French]. Ann Chir Plast Esthet 2006;51 (4-5) 339- 346
PubMed Link to Article
Tille  JCMorris  MABrundler  MAPepper  MS Familial predisposition to tufted angioma: identification of blood and lymphatic vascular components. Clin Genet 2003;63 (5) 393- 399
PubMed Link to Article
Lyons  LLNorth  PEMac-Moune Lai  FStoler  MHFolpe  ALWeiss  SW Kaposiform hemangioendothelioma: a study of 33 cases emphasizing its pathologic, immunophenotypic, and biologic uniqueness from juvenile hemangioma. Am J Surg Pathol 2004;28 (5) 559- 568
PubMed Link to Article
Lam  WYMac-Moune Lai  FLook  CNChoi  PCAllen  PW Tufted angioma with complete regression. J Cutan Pathol 1994;21 (5) 461- 466
PubMed Link to Article
Chu  CYHsiao  CHChiu  HC Transformation between kaposiform hemangioendothelioma and tufted angioma. Dermatology 2003;206 (4) 334- 337
PubMed Link to Article
Okada  ETamura  AIshikawa  OMiyachi  Y Tufted angioma (angioblastoma): case report and review of 41 cases in the Japanese literature. Clin Exp Dermatol 2000;25 (8) 627- 630
PubMed Link to Article
Ward  KAKennedy  CTAshworth  MT Acquired tufted angioma frequently develops at sites other than the neck and upper trunk. Clin Exp Dermatol 1996;21 (1) 80
PubMed Link to Article
Kamath  GHBhat  RMKumar  S Tufted angioma. Int J Dermatol 2005;44 (12) 1045- 1047
PubMed Link to Article
Ishikawa  KHatano  YIchikawa  HHashimoto  HFujiwara  S The spontaneous regression of tufted angioma: a case of regression after two recurrences and a review of 27 cases reported in the literature. Dermatology 2005;210 (4) 346- 348
PubMed Link to Article
Descours  HGrézard  PChouvet  BLabeille  B Acquired tufted angioma in an adult [in French]. Ann Dermatol Venereol 1998;125 (1) 44- 46
PubMed
Barco  DBaselga  ERibé  AAlomar  A Congenital self-limiting tufted angioma [in Spanish]. Actas Dermosifiliogr 2008;99 (5) 423- 425
PubMed Link to Article
Enjolras  OWassef  MDosquet  C  et al.  Kasabach-Merritt syndrome on a congenital tufted angioma [in French]. Ann Dermatol Venereol 1998;125 (4) 257- 260
PubMed
Satter  EKGraham  BSGibbs  NF Congenital tufted angioma. Pediatr Dermatol 2002;19 (5) 445- 447
PubMed Link to Article
Igarashi  MOh-i  TKoga  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 2000;27 (8) 537- 542
PubMed
Kasabach  HHMerritt  KK Capillary hemangioma with extensive purpura: report of a case. AJDC 1940;591063- 1070
Haisley-Royster  CEnjolras  OFrieden  IJ  et al.  Kasabach-Merritt phenomenon: a retrospective study of treatment with vincristine. J Pediatr Hematol Oncol 2002;24 (6) 459- 462
PubMed Link to Article
Brasanac  DJanic  DBoricic  IJovanovic  NDokmanovic  L Retroperitoneal kaposiform hemangioendothelioma with tufted angioma-like features in an infant with Kasabach-Merritt syndrome. Pathol Int 2003;53 (9) 627- 631
PubMed Link to Article
Hall  GW Kasabach-Merritt syndrome: pathogenesis and management. Br J Haematol 2001;112 (4) 851- 862
PubMed Link to Article

Figures

Place holder to copy figure label and caption
Figure 1.

Wide tufted angioma and Kasabach-Merritt syndrome in patient 11 at age 6 months involving genitalia area (A) and the back (B).

Graphic Jump Location
Place holder to copy figure label and caption
Figure 2.

Congenital abdominal infiltrating tufted angioma in patient 4. A, In the newborn “quiescent” state. B, The same lesion at age 2 years is inflammatory and painful, with biologic coagulopathy.

Graphic Jump Location
Place holder to copy figure label and caption
Figure 3.

Typical tufted angioma. A, Tufted angioma is characterized by round small lobules scattered in the mid and deep dermis and superficial subcutaneous tissue and in a cannonball distribution (hematein-eosin–saffron [HES], original magnification ×25). B, Typical appearance of tufts in tufted angioma with closely packed capillaries and empty crescent-shaped vessels encircling them (HES, original magnification ×100). Fibrosis is obvious in the surrounding dermis. C, High magnification shows the center of a lobule (HES, original magnification ×400). Some areas closely resemble kaposiform hemangioendothelioma or Kaposi sarcoma and show spindle cells separated by slitlike lumina containing few red blood cells (star). Some hyaline globules can also been seen (arrowhead). Besides these capillary tufts, some larger vessels with a thin wall and an empty lumen can be seen (arrow). D, Staining with D240 antibody (podoplanin) shows evidence of partial lymphatic differentiation. E, Another tufted angioma shows larger and ill-defined tufts having a tendency to coalesce, also associated with anastomosed lymphatic vessels (HES, original magnification ×25).

Graphic Jump Location

Tables

Table Graphic Jump LocationTable 1. Characteristics of 13 Patients With Tufted Angioma (TA)
Table Graphic Jump LocationTable 2. Key Differences Between Tufted Angioma and Superficial Kaposiform Hemangioendothelioma
Table Graphic Jump LocationTable 3. Comparison of the Present Series With Previously Published Studies

References

Nakagawa  K Case report of angioblastoma of the skin [in Japanese]. Jpn J Dermatol 1949;5992- 94
MacMillan  AChampion  RH Progressive capillary haemangioma. Br J Dermatol 1971;85 (5) 492- 493
PubMed
Jones  EW Dowling Oration 1976: malignant vascular tumours. Clin Exp Dermatol 1976;1 (4) 287- 312
PubMed Link to Article
Herron  MDCoffin  CMVanderhooft  SL Tufted angiomas: variability of the clinical morphology. Pediatr Dermatol 2002;19 (5) 394- 401
PubMed Link to Article
Wong  SNTay  YK Tufted angioma: a report of five cases. Pediatr Dermatol 2002;19 (5) 388- 393
PubMed Link to Article
Browning  JFrieden  IBaselga  EWagner  AMetry  D Congenital, self-regressing tufted angioma. Arch Dermatol 2006;142 (6) 749- 751
PubMed Link to Article
Jang  KAChoi  JHSung  KJMoon  KCKoh  JK Congenital linear tufted angioma with spontaneous regression. Br J Dermatol 1998;138 (5) 912- 913
PubMed Link to Article
Jones  EWOrkin  M Tufted angioma (angioblastoma): a benign progressive angioma, not to be confused with Kaposi's sarcoma or low-grade angiosarcoma. J Am Acad Dermatol 1989;20 (2, pt 1) 214- 225
PubMed Link to Article
Teillac-Hamel  DAndry  PBodemer  C  et al.  Kasabach-Merritt syndrome in children [in French] Ann Pediatr (Paris) 1992;39 (7) 435- 441
PubMed
Enjolras  OWassef  MMazoyer  E  et al.  Infants with Kasabach-Merritt syndrome do not have “true” hemangiomas. J Pediatr 1997;130 (4) 631- 640
PubMed Link to Article
Enjolras  OMulliken  JBWassef  M  et al.  Residual lesions after Kasabach-Merritt phenomenon in 41 patients. J Am Acad Dermatol 2000;42 (2, pt 1) 225- 235
PubMed Link to Article
Weiss  SWGoldblum  JR Enzinger and Weiss' Soft Tissue Tumors. 5th New York, NY Elsevier Inc2008;
Enjolras  OPicard  ASoupre  V Congenital haemangiomas and other rare infantile vascular tumours [in French]. Ann Chir Plast Esthet 2006;51 (4-5) 339- 346
PubMed Link to Article
Tille  JCMorris  MABrundler  MAPepper  MS Familial predisposition to tufted angioma: identification of blood and lymphatic vascular components. Clin Genet 2003;63 (5) 393- 399
PubMed Link to Article
Lyons  LLNorth  PEMac-Moune Lai  FStoler  MHFolpe  ALWeiss  SW Kaposiform hemangioendothelioma: a study of 33 cases emphasizing its pathologic, immunophenotypic, and biologic uniqueness from juvenile hemangioma. Am J Surg Pathol 2004;28 (5) 559- 568
PubMed Link to Article
Lam  WYMac-Moune Lai  FLook  CNChoi  PCAllen  PW Tufted angioma with complete regression. J Cutan Pathol 1994;21 (5) 461- 466
PubMed Link to Article
Chu  CYHsiao  CHChiu  HC Transformation between kaposiform hemangioendothelioma and tufted angioma. Dermatology 2003;206 (4) 334- 337
PubMed Link to Article
Okada  ETamura  AIshikawa  OMiyachi  Y Tufted angioma (angioblastoma): case report and review of 41 cases in the Japanese literature. Clin Exp Dermatol 2000;25 (8) 627- 630
PubMed Link to Article
Ward  KAKennedy  CTAshworth  MT Acquired tufted angioma frequently develops at sites other than the neck and upper trunk. Clin Exp Dermatol 1996;21 (1) 80
PubMed Link to Article
Kamath  GHBhat  RMKumar  S Tufted angioma. Int J Dermatol 2005;44 (12) 1045- 1047
PubMed Link to Article
Ishikawa  KHatano  YIchikawa  HHashimoto  HFujiwara  S The spontaneous regression of tufted angioma: a case of regression after two recurrences and a review of 27 cases reported in the literature. Dermatology 2005;210 (4) 346- 348
PubMed Link to Article
Descours  HGrézard  PChouvet  BLabeille  B Acquired tufted angioma in an adult [in French]. Ann Dermatol Venereol 1998;125 (1) 44- 46
PubMed
Barco  DBaselga  ERibé  AAlomar  A Congenital self-limiting tufted angioma [in Spanish]. Actas Dermosifiliogr 2008;99 (5) 423- 425
PubMed Link to Article
Enjolras  OWassef  MDosquet  C  et al.  Kasabach-Merritt syndrome on a congenital tufted angioma [in French]. Ann Dermatol Venereol 1998;125 (4) 257- 260
PubMed
Satter  EKGraham  BSGibbs  NF Congenital tufted angioma. Pediatr Dermatol 2002;19 (5) 445- 447
PubMed Link to Article
Igarashi  MOh-i  TKoga  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 2000;27 (8) 537- 542
PubMed
Kasabach  HHMerritt  KK Capillary hemangioma with extensive purpura: report of a case. AJDC 1940;591063- 1070
Haisley-Royster  CEnjolras  OFrieden  IJ  et al.  Kasabach-Merritt phenomenon: a retrospective study of treatment with vincristine. J Pediatr Hematol Oncol 2002;24 (6) 459- 462
PubMed Link to Article
Brasanac  DJanic  DBoricic  IJovanovic  NDokmanovic  L Retroperitoneal kaposiform hemangioendothelioma with tufted angioma-like features in an infant with Kasabach-Merritt syndrome. Pathol Int 2003;53 (9) 627- 631
PubMed Link to Article
Hall  GW Kasabach-Merritt syndrome: pathogenesis and management. Br J Haematol 2001;112 (4) 851- 862
PubMed Link to Article

Correspondence

CME
Also Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
Your answers have been saved for later.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
Submit a Comment

Multimedia

Some tools below are only available to our subscribers or users with an online account.

Web of Science® Times Cited: 16

Related Content

Customize your page view by dragging & repositioning the boxes below.

Articles Related By Topic
Related Collections
PubMed Articles