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On-line version ISSN 1806-4841
An. Bras. Dermatol. vol.81 suppl.3 Rio de Janeiro Sept./Oct. 2006
Tufted angioma: a case report treated with pulsed-dye laser*
Alberto Oiticica CardosoI; Tatiana TaytiI; Juliana Pedroso de OliveiraII; Luís Antonio TorezanII; Mirian N. SottoIII; Maria Aparecida VilelaIII; Marcello Menta Simonsen NicoIII; Nuno de Sanches OsórioII; Cyro Festa NetoIV
IResident doctor/trainee in Dermatology
at Hospital das Clínicas da Faculdade de Medicina da Universidade de
São Paulo - HC-FMUSP - São Paulo (SP), Brazil
IIIPhD, Prof at the Departament of Dermatology at Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo - HC-FMUSP - São Paulo (SP), Brazil
IVProf at the Departament of Dermatology at Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo - HC-FMUSP - São Paulo (SP), Brazil
Tufted angioma is a rare, benign, cutaneous angiomatous proliferation. It is more common in children and is clinically characterized by red-purple painfull plaques, more common in trunk. When spontaneous regression does not occur, proposed treatments (conventional surgery, steroid therapy, interferon and laser) achieve little success. We describe a case of a 11-year-old girl with a tufted angioma located in the trunk treated with pulsed-dye laser, showing important relief of local pain, and small reduction in lesion size. We have been motivated to describe this case due to the small number of such reports in Brazil and by the attempt to treat it with pulsed-dye laser.
Keywords: Hemangioma; Lasers; Therapeutics
Tufted angioma is a rare, benign condition of cutaneous angiomatous proliferation whose name is based on its histological pattern. It affects children more often, and is clinically characterized by pain erithematous-purple plaque lesions, more frequent in trunk.1 Its diagnosis is made by characteristic histological examination, with the presence of vessels grouped in glomerulus-like patterns with the aspect of a "cannon bullet".2 When it does not regress spontaneously, it responds little to proposed treatments, such as conventional surgery, steroids, interferon and laser.
Approximately 60 cases have been described in the English literature and 160 in Japan, where it is called angioblastoma.3 The existence of rare cases in Brazil4 and the attempt of treatment with pulsed-dye laser have motivated the publication of the case.
Eleven-year-old brown-skinned female patient, Born at and coming from Nova Conquista, Bahia, who sought for our service with the complaint of a spot in the right mammary region, with progressive growth for six years. Upon dermatological examination, an erithematous-purple infiltrated plaque was noted on the right mammary region, together with multiple satellite plaques with the same features, extending to the dorsum (Figure 1). Lesions presented an increase in local sensitivity and were painful to palpation, and had no history of bleeding or ulceration.
A biopsy of the mammary lesion was performed, with the observation, on histological examination, of vessels grouped in glomerulus-like patterns with the aspect of a "cannon bullet" in the mid and deep reticular dermis. In the periphery of the tufted capillaries, were noted canalicular structures in glomerulus-like patterns (Figure 2) with thin dilated walls, with a semilunar aspect. Upon immunhistochemistry, dermal dendrocytes (CD34+) were observed, evidencing the "cannon bullet" pattern. Histogical findings were compatible to the diagnosis of tufted angioma.
With the intention of verifying the extension of the lesions, a magnetic resonance was carried out, revealing the presence of a hypervascular disc-shaped lesion affecting skin and underlying subcutaneous tissue of the anteroposterior wall of the right thorax, with no signs of infiltration in the underlying muscles.
An initial attempt was made for a treatment with intralesional injection of trianciolone at 5mg/mL; however, the patient complained a lot of pain, making the procedure impossible.
Based on the literature and on the vascular nature of the pathology, the choice was made for the attempt of treatment with laser. Initially a test with NdYAG long pulse laser (Vasculight®/Lumenis, 1064nm of wavelength) was carried out, with a fluence of 90J and pulse duration of 100 ms, with no response. Later, pulsed-dye laser was attempted (Candela-VBEAM FLPDL, with 585 to 595nm of wavelength). Patient was submitted to four sessions, in which large areas were treated, with the fluence ranging from 8,0 to 9,0J/cm2, a spot of 7mm, and pulse duration time ranging from 0,45 to 3ms. Interval between sessions was 45 days. During laser application, the patient complained of pain, despite use of topic local anesthetic (lidocaine and prilocaine). Lesion size reduced little and, by the end of the sessions, there was a clinical improvement of about 20% of treated areas (Figure 3), yet with an almost total decrease in pain. During case follow-up, of around three years, new papules appeared, demonstrating the case was not yet stabilized.
Tufted angioma was described by Nakagawa5 n 1949 under the name of angioblastoma. Later, Wilson-Jones,2 in 1976, based on a case series, designated this vascular tumor as an "acquired tufted angioma", with regards to its histological aspect. They were initially considered to be distinct nosological entities, but today many consider them as the same disease.6,7 There are reports of this vascular tumor in Asia, Europe, United States and Latin America.
Tufted angioma is a rare, benign condition of cutaneous angiomatous proliferation. Mechanism leading such vascular proliferation remains obscure. Several hypotheses have been formulated, based on clinical observations. The possible participation of estrogens was considered in a patient whose tumor onset happened in two consecutive pregnancies, disappearing after delivery.7 Other possibilities are post-traumatic hyperplasic vascular reaction or alteration in supporting factor of angiogenesis, as observed in a patient who had received liver transplantation with ensuing spontaneous development of lesions.8 In the present case, as in most cases, the condition appeared spontaneously, with no apparent triggering factor.
Tufted angioma is clinically characterized by the presence of stains, papules and plaques of red-violet color, with a tendency to increase in size, many times very quickly, to then stabilize. Spontaneous regression may then follow.7 It is generally located in trunk and limbs.2
Onset of tufted hemangioma usually occurs in the first two decades of life, specially during the first year. Other clinical findings include increase in local sensitivity, and, more rarely, hyperhydrosis and hypertricosis.9 All clinical and epidemiological data pof the present patient agree to those in literature.
In the case of tufted hemangioma in children, differential diagnosis is clinically made with childhood hemangioma, which is usually soft and not painful to palpation, and, more rarely, with hemangioendothelioma, infantile hemangiopericytoma and infantile myofibroma.9 When in adults, vascular tumors such as Kaposi's sarcoma and angiosarcoma should be discarded.10
Diagnosis is generally based on the histological picture, very typical in tufted hemangioma, presenting a lobular pattern of densely distributed capillaries with the aspect of a "cannon bullet".2 Vascular tufts are constituted by long hypertrophied endothelial cells, very next one to the other, which makes it hard to define the lumen of the capillary. Endothelial cell nuclei are regular, round, egg-shaped or fusiform. This allows differentiation from other vascular pathologies with relative safety. Immunohistochemistry (CD 34+) is not necessary for diagnostic confirmation, even though it helps in the confirmation of the vascular origin.
There are few reports of effective treatments for tufted hemangioma in the literature. Apparently, surgical excision, when feasible, is the best option, wit high success and low relapse rates.2 In the present case, tumor extension was a contra-indication for such a procedure.
Other options are steroids, both systemically in high doses and topically (clobetasol propionate at 0,05%),6 with little size reduction of the lesions, and some improvement in pain. Attempts to make intralesional steroid infiltration, in this case, were not unviable due to local pain produced, making treatment evaluation impossible.
Use of a-interferon, either systemically or intralesionally, is still controversial, either with good or no therapeutical results.10
Pulsed-dye laser promotes selective vascular destruction with minimal aggression to surrounding skin. Thus, capillary tufts in these case could serve as a target for the laser.11 Even though this is a rare disease, five cases of treatment with pulsed-dye laser for tufted hemangioma have been described, with variable results in two of them with good response,11,12 and with complete failure in three others.1,13,14 Yet, because it is a relatively safe method, with few side effects, the main being post-inflammatory hyperpigmentation, which regresses later on,15 such therapy was chosen.
The use of pulsed-dye laser was efficient in reducing pain, but the same success was not obtained with lesion size, in spite of the slight improvement. As there are few reports of treatment with pulsed-dye laser, it is still early to state a clear opinion on the indication of such laser for the treatment of tufted angioma. However, it can be stated that the improvement in pain justified its use. In this case, there were no side effects, only pain during application of the method, which reinforces its safety.
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Alberto Eduardo Oiticica Cardoso
Rua Ponta Delgada, 76 - Apto 182 - Vila Olímpia
04548-020 - São Paulo - SP - Brazil
Tel.: +55 11 8147-8946
Received on April 06, 2004.
Approved by the Consultive Council and accepted for publication on May 29, 2006.
* Work done
at the University Hospital of the Universidade de São Paulo - HC-FMUSP
- São Paulo (SP), Brazil.
Conflict of interests: None