Indurated Dusky Red Swelling on the Forearm of an Infant: Tufted Angioma

Abstract

Tufted angioma (TA) is a rare benign vascular tumor that may be congenital or acquired and generally presents as an asymptomatic or painful solitary erythematous to violaceous poorly defined plaque. Hyperhidrosis and hypertrichosis may be associated. The lesions have tendency to resolve spontaneously, in majority, within 2 years of disease onset. However, occurrence of Kassbach-Merritt phenomenon should be looked for. We present a case of 8-months-old infant with TA over the right forearm with classical clinical, histoptahological and immunohistochemistry features.

Introduction

Tufted angioma (TA) is a benign vascular tumor which presents as an erythematous to violaceous plaque with hypertrichosis and hyperhidrosis.[1] The diagnosis is suspected on typical history and clinical examination; and confirmed on histopathology and immunohistochemistry markers like CD31, CD34 and smooth muscle actin. Kassabach-Merritt phenomenon (KMP) is a possible rare complication and should be regularly monitored.[1,2] We present a case of TA with typical clinical and histological findings along with specific immunohistochemistry markers.

Case Report

An 8-month-old female infant was brought to the dermatology department with dusky red swelling over the right forearm. She was born of nonconsanguineous marriage at term by normal vaginal delivery. The swelling started as a pinkish-red patch at 1 month of age and progressively increased to the present size over the next 7 months. Parents noticed excessive sweating and increased hair growth over the lesion. Physical health of the infant was otherwise normal with no history of bleeding from any site.

Examination revealed a single dusky red to violaceous, ill-defined indurated plaque involving most of the forearm, predominantly the ventral aspect [Figure 1]. It was nonblanchable and tender to touch with raised temperature as compared to the surrounding normal skin. Hyperhidrosis and hypertrichosis were noticeable over the margins. No other systemic abnormality was observed on examination.

Figure 1.

Dusky red-to-violaceous, ill-defined indurated plaque involving most of the forearm. Note hypertrichosis at the margins of the plaque

Hematological and biochemical parameters of the infant including the coagulation profile were within normal limits. Roentogram of the limb showed soft-tissue swelling with no bony abnormality. Ultrasound abdomen and pelvis was noncontributory. The skin biopsy from the lesion on hematoxylin- and eosin-stained sections demonstrated the presence of discrete lobules or tufts of capillaries scattered in the superficial and deep dermis. The capillary lobules were highly cellular and densely packed with vascular spaces [Figure 2]. Immunohistochemistry was positive for CD34, smooth muscle actin, and vimentin confirming its vascular origin [Figures 3, 4a and b]. Based on the morphological and histological features and findings on Immunohistochemistry, a diagnosis of tufted angioma (TA) in the infant was made.

Figure 2.

Histopathology showing discrete circumscribed lobules or tufts of capillaries scattered in the superficial and deep dermis. The capillary lobules are highly cellular and densely packed with scanty vascular spaces (H and E, ×40)

Figure 3.

Immunohistochemistry showing positivity of capillary lobules for CD34 (immunohistochemistry, ×400)

Figure 4.

(a) Immunohistochemistry positivity of capillary lobules for smooth muscle actin confirming its vascular origin (immunohistochemistry, ×400). (b) Capillary lobules showing positivity for vimentin (immunohistochemistry, ×400)

Parents were counseled regarding the benign nature of the lesion. Hence, in consultation with the parents, a conservative approach was adopted. She was being followed up at a 6-week interval. At 6-month follow up, the child was found to be stable, and a decrease in the size of swelling was observed.

Discussion

TA is a rare benign vascular tumor with varied clinical presentation and disease progression. It can be congenital or acquired; the frequency of congenital TA being 54%.[1] Acquired cases have early childhood onset in majority. TA generally presents as a solitary erythematous to violaceous poorly defined infiltrated plaque often associated with pain, hyperhidrosis, and hypertrichosis.[1] Initial slow growth phase of the lesion lasts for few months and is followed by a phase of stabilization. Acquired TA tends to have comparatively longer slow-growth phase and is less likely to regress spontaneously. In a review of 27 cases of congenital TA, spontaneous regression was reported in 95% of cases within 2 years of disease onset.[2] Kasabach–Merritt phenomenon (KMP) occurs in approximately 10% of cases and should be suspected if a lesion displays sudden increase in size and becomes painful.[1] KMP is characterized by the triad of consumptive coagulopathy, microangiopathic hemolytic anemia, and rapid enlargement of tumor. Recurrent episodes of KMP with TA have also been reported.[3]

Pathogenesis of TA is still unclear. Increased local secretion of growth factors such as interleukin-8, stimulating vasculogenesis, and promoting vascular lobule formation has been reported.[4] Histopathology of TA was first described in detail by Jones in 1976. It shows small tufts of capillaries and angiomatous lobules in the dermis with dilated lymphatic channels with characteristic “cannonball” appearance.[5] Immunohistochemical markers include positive CD31, CD34, and smooth muscle actin. Recently, the vascular lobules are found to be D2-40 and Prox 1 positive which points toward its partial lymphatic nature.[5]

Imaging studies such as ultrasonography or magnetic resonance imaging are employed to determine the extent and depth of the lesion. Hematological studies including the coagulation profile are performed to exclude KMP.

The differential diagnoses of TA include infantile and childhood hemangioma, vascular malformation, Kaposi sarcoma, and kaposiform hemangioendothelioma (KHE). A new classification by the International Society for the Study of Vascular Anomalies has separated TA and KHE into benign vascular tumors and locally aggressive vascular tumors, respectively.[6]

Distinction from Kaposi sarcoma, angiosarcoma, capillary hemangioma, and KHE is important. Endothelial cells in TA may sometimes show slight spindling, but the elongated spindle cells are characteristic of Kaposi sarcoma.[7] The main distinguishing feature from angiosarcoma is the absence of cell atypia.[7] Capillary hemangioma shows proliferation of endothelial cells forming lobular arrangement of well-formed capillaries, but the clusters of endothelial cells in TA are larger and more irregular. Furthermore, the “cannonball” distribution of nodules is typically seen in TA.[8] KHE shares most of the clinical and histological features of TA. KHE exhibits spindled endothelial cells with slit-like vessels with growth of these cells in sheets or coalescing nodules rather than dispersed tufts as observed in TA.[1,9]

There is no consensus on the management of TA till date. Various factors such as location, functional compromise, size, pain, secondary changes, and KMP help in individualizing the treatment. As in our case, a conservative approach could be adopted for an asymptomatic, uncomplicated lesion with minimal or no risk of development of KMP.[2]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.