Acquired multifocal tufted angiomas in an immunocompetent young adult.

Tufted angioma (TA) is a rare benign vascular neoplasm, localized to the skin and subcutaneous tissues, occurring primarily on the trunk and extremities of children. The lesions are usually asymptomatic but, rarely, paroxysmal painful episodes may be associated. The occurrence of eruptive TA is still rarer and had been described almost exclusively in association with immunocompromised states. We report here a case of acquired painful multifocal tufted angiomas on the face and neck in an immunocompetent young adult.

Introduction

Tufted angioma (TA) is a rare vascular neoplasm localised to the skin and subcutaneous tissues, occurring primarily on the trunk and extremities of children.[1] These tumours pursue a benign course of slow angiomatous proliferation, without any evidence of aggressive behaviour or metastases.[2] TA shows equal gender distribution without any racial predilection. The lesions are usually asymptomatic but, rarely, paroxysmal painful episodes may also be associated with it.[3] The occurrence of eruptive TA is still rarer, and has been described almost exclusively in association with immunocompromised states.[4] We report here a case of acquired painful multifocal tufted angiomas on the face and neck in a young adult.

Case Report

A 19-year-old man presented with multiple, reddish, elevated, and painful skin lesions on the left side of his face and neck for the duration of three months. There was no history of trauma, bleeding from the lesions or from other parts of the body, or increased sweating or discharge from the lesion. There was no history of similar illness in the family. Examination revealed a few erythematous plaques on his left cheek [Figure 1]. Papules of different sizes were scattered on and around the plaques. In addition, multiple small erythematous nodules (measuring 1 × 1 cm) were present on his upper neck [Figure 2] for the same duration. The lesions were tender, firm in consistency with a slight rise in local temperature. Other parts of the skin, nails, hair, and mucosae were normal. Systemic examination was non-contributory. Punch biopsy specimens of the skin from a facial plaque and a nodule on the neck showed multiple, discrete, circumscribed dermal foci of closely set vascular channels. The lobules consisted of multiple capillaries with bloodless lumen, surrounded by dilated crescent-shaped vascular channels [Figure 3]. In addition, there was increased sebaceous gland proliferation in the dermis. There was no cellular atypia or mitotic figures. Complete hemogram, prothrombin time, partial thromboplastin time, bleeding time, clotting time and other routine biochemical panels showed normal results. Screening for HIV infection was negative. X-ray chest and ultrasonography of the abdomen were normal. Based on the clinical and histopathological features, a diagnosis of tufted angioma was made. The patient was treated with fluticasone propionate ointment, which reduced the painful episodes to some extent, but the lesions were persistent.

Figure 1

Multiple erythematous plaques and papules on left cheek

Figure 2

Multiple erythematous plaques and papules and small erythematous nodules on upper neck

Figure 3

(a) Multiple, discrete, circumscribed dermal foci of closely set vascular channels (H and E staining, ×100). (b) Multiple capillaries with bloodless lumen, surrounded by dilated crescent - shaped vascular channels resembling a "cannonball" (H and E staining, ×400)

Discussion

Most cases (60-70%) of TA develop before the age of five years and fewer than 10% of cases with TA had occurred after the age of 50 years. The lesion of TA enlarges slowly over 5 months to 10 years, after which no further growth occurs. Although multiple lesions may occur. TA usually presents as solitary nodule or papule.[2] Clinically, TA appears as a deep red or purple patch or plaque with superimposed angiomatous papules that predominantly appear on the upper thorax, neck, and shoulders. Face, scalp, and proximal extremities are rarely involved. The diameter of the patch or plaque generally ranges from a few millimeters to several centimeters. On palpation, the lesions often have a rubbery consistency, and may be tender. Peripheral extension occurs occasionally, and lesions of TA are rarely described as having a thick indurated border and a central depression (annular TA).[5] Hypertrichosis may also be seen in association with TA.[6] Occasionally, TA may be associated with the Kasabach-Merritt syndrome (KMS).[2] In this setting, the presence of petechial and ecchymotic patches should alert the physician to the development of KMS. No specific laboratory study is helpful in the diagnosis or treatment of TA.[2] On suspecting the coexistence of KMS, a complete haemogram (including a platelet count), prothrombin time and/or activated partial thromboplastin time, and full screening for disseminated intravascular coagulation is indicated. Magnetic resonance imaging (MRI) studies have proven useful in the evaluation of the depth of invasion and extent of growth of TA.[2] TAs have a specific and distinctive histopathological pattern. They are characterised by the vascular tufts of densely packed capillaries, randomly scattered throughout the dermis in a so-called “cannonball distribution.”[5] There are crescentic spaces surrounding the vascular tufts and lymphatic-like spaces within the tumour stroma. The epidermis is normal in most cases. The surrounding cutaneous appendageal structures of the dermis usually remain uninvolved. No oedema or inflammation surrounds the vascular lobules.

Partial spontaneous regression of TA may occur, but complete disappearance is extremely rare. No causes of TA have yet been established. Skin lesions usually occur without a history of preceding trauma. TA has also been reported to occur after and during pregnancy.[7] Two previous reports of acquired eruptive TA exist in the Pubmed database. In one patient, eruptive TA occurred after liver allograft transplant[8] and, in the other, it appeared in a patient with Crohn's disease, who was receiving immunosuppressants.[4] In these cases, immunosuppression or influence of drugs on angiogenesis might have played a role in the development of TA. However, no such association was found in our patient. TA should be differentiated from Kaposiform haemangioendothelioma, juvenile capillary, or strawberry angioma, haemangiopericytoma, glomeruloid haemangioma, lobular capillary haemangioma, or pyogenic granuloma.

Different modalities of treatment have been tried for TA. These include complete surgical excision, cryosurgery, and radiotherapy.[4] Pulsed dye laser has also been reported as an effective option for treatment of TA.[9] Potent topical steroids have been used to reduce pain.[3] High doses of interferon α2[10] and systemic steroids[11] may be tried before proceeding to excision, particularly in cases with extensive lesions.

The case is reported here for the combination of rare clinical features and to emphasise the importance of considering TA in the differential diagnoses of any acquired vascular tumour.