Multifocal Annular Tufted Angioma: An Uncommon Clinical Entity.

Tufted angioma (TA) is a localized benign hamartomatous vascular proliferation usually presenting in the childhood as an erythematous plaque. We report here a rare case of multifocal TA in an 8-year-old boy who presented which two large annular lesions as well as multiple papules and nodules on the back for the duration of 4 years. Histology showed typical well circumscribed poorly canalized vascular lobules with 'cannon ball' configuration.

What was known?

Tufted angioma is a rare benign vascular neoplasm of childhood typically presenting with erythematous plaques or nodules showing tufts of vascular proliferation on histology.

Introduction

Tufted angioma is a rare vascular neoplasm clinically characterized by slowly progressive pink to red macules or plaques on the neck, trunk, or shoulders of children or young adults. These tumors follow a benign course of angiomatous proliferation, without any evidence of malignancy. TA shows neither gender nor racial preferences. We report herein a case of multifocal tufted angiomas with annular morphology in a child for the rarity of the condition.

Case Report

An 8-year-old boy was referred to us for the evaluation of multiple reddish elevations on the back, which were first noticed 4 years before presentation. Starting as two small reddish asymptomatic patches, the lesions gradually became elevated and fresh lesions appeared in the vicinity of the older ones. The lesions have remained stable for the preceding several months. Increased sweating over the patches was noted by the parents. There was no history of bleeding from the lesions or any other body site. Family history and past medical history was unremarkable.

On examination, two brightly erythematous annular plaques of about 6 cm and 3 cm diameter were seen over the left lateral portion of the back. The lesions were well-marginated with irregular borders [Figure 1]. In addition, several erythematous papules and plaques of varying sizes were seen around the lesions as well as on the right side of the back. The lesions were non-tender, and seemed to be moist. No other systemic or cutaneous abnormalities were detected. Routine laboratory tests, blood coagulation profile, and serum biochemistry panel were within normal limits.

Figure 1

Two annular erythematous plaques and several papules and plaques of varying sizes on the back

We considered the differential diagnoses of tufted angioma, sudoriferous angioma (eccrine angiomatous hamartoma), and capillary hemangioma. A punch biopsy specimen was obtained from a plaque. Histopathological examination revealed circumscribed, scattered foci of closely set capillaries in the dermis. These discrete, ovoid angiomatous lobules or tufts had a “cannonball” appearance. Crescent-shaped cleft like spaces were seen around the lobules [Figures 2 and 3]. The lobules were highly cellular with scanty vascular spaces within. There was no increase in eccrine sweat gland structures. Based on the clinical findings and characteristic histology, a diagnosis of tufted angioma was made. After discussion about the possible modalities of treatment and the chance of spontaneous regression, the parents preferred regular follow-up without any active intervention and the child was kept under observation.

Figure 2

Histopathology showing well-circumscribed poorly-canalized capillary lobules in the dermis with crescent-shaped spaces around them. H and E, ×100

Figure 3

Showing well-defined capillary tufts surrounded by crescent-shaped clefts. H and E, ×400

Discussion

TA was described for the first time by Wilson Jones in 1976 as an acquired vascular proliferation of peculiar histological characteristics and reports of additional cases had helped better characterization of this clinicopathologic entity.[12] Tufted angioma (TA) can be congenital or acquired. It commonly presents in infancy or early childhood, though adult-onset cases have been reported also.[34] TA usually presents as a macule, papule or plaque over the upper trunk, neck and proximal part of the limbs; involvement of other locations like face, oral mucosa and lip is also known.[5] TA is a sporadic disease with more than half of the cases occurring during the first 5 years of life. However, familial cases have also been reported.[6] The lesions are usually asymptomatic but, rarely, paroxysmal painful episodes may occur. Tenderness is a usual feature, which was absent in our case. Hyperhidrosis, as in our case, and hypertrichosis can also occur over the lesion.[7] Eccrine sweat gland elements however were not seen to be increased in the histopathology of our patient.

TA should be differentiated from Kaposiform hemangioendothelioma, juvenile capillary, or strawberry angioma, haemangiopericytoma, glomeruloid haemangioma, or pyogenic granuloma.[3] TA can however be diagnosed by the distinctive histopathological pattern characterized by the vascular tufts of densely packed capillaries, randomly scattered throughout the dermis in a so called “cannonball” configuration. There are crescentic spaces surrounding the vascular tufts and lymphatic-like spaces within the tumor stroma.[8] The epidermis is normal in most cases. The surrounding cutaneous appendageal structures of the dermis usually remain uninvolved. Immunohistochemical stains show strong positivity for Ulex uropaeus I Lectin and EN4 and unlike infantile hemangioma, negative staining for GLUT 1.

TA, like Kaposiform hemangioendothelioma, may be associated with the Kasabach-Merritt syndrome (KMS). Physicians should look for the appearance of petechiae or ecchymotic patches and if they occur, a complete hemogram (including a platelet count), prothrombin time and/or activated partial thromboplastin time and full screening for disseminated intravascular coagulation should be ordered.[3] Common association with KMS, morphologic similarity, association in the same patient, and common expression of lymphatic markers had led to the suggestion that TA may be a minor form of Kaposiform hemangioendothelioma or part of a spectrum of the same condition.[9]

TA usually pursue a persistent course, but spontaneous regression may occur in a proportion of cases.[10] Different modalities of treatment including complete surgical excision, cryosurgery, and radiotherapy had been tried for TA. Pulsed dye laser has also been reported as an effective option for treatment of TA.[11] Embolization and vincristine are found to be effective in patients having KMS.[12]

In conclusion, we have reported a rare case of multiple tufted angiomas with annular morphology in a child. The possibility of this tumor should be kept in mind while dealing with patients having lesions of similar morphology.

What is new?

We described a boy who had multifocal lesions with large annular plaques as well as papules and nodules.