Tufted angioma.

Sir,

An 18-year-old female presented with multiple red raised lesions over the neck since the age of eight years. There was no history of trauma, bleeding from the lesions, or excessive sweating. There was no history of similar complaints in the family. Cutaneous examination revealed multiple erythematous and skin-colored plaques on the neck [Figure 1]. The lesions were firm, nontender, noncompressible, and warm on palpation. There was no thrill or bruit over the plaque. Systemic examination did not reveal any abnormality.

Figure 1

Erythematous papules and nodules on the neck

Skin biopsy from the nodule revealed clusters of numerous dilated, thin- and thick-walled capillaries within the dermis [Figures 2 and 3]. The capillaries were lined by plump or elongated endothelial cells. Some of the capillaries with thick walls were engorged with RBCs. The dermis surrounding these clusters revealed fibroplasia. Overlying epidermis showed mild hyperplasia.

Figure 2

Clusters of dilated capillaries in the dermis (hematoxylin and eosin ×10)

Figure 3

Dilated capillaries (hematoxylin and eosin ×40)

Tufted angioma (TA) is a rare, benign angiomatous condition. It is also called angioblastoma of Nagakawa. It affects both the genders equally. The lesions usually arise between the ages of 1 and 5 years. Occasionally, the lesions may be present at birth.[1] They may rarely develop in adults or in old age. Partial spontaneous regression of TA may occur, but complete disappearance is extremely rare.[2]

Clinically, the lesions are characterised by erythematous papules, plaques, and nodules. Atypical presentations of annular plaques may be seen. The most commonly involved sites are neck, upper trunk, and proximal part of extremities.[2] The lesions may show increased sweating, or growth of fine lanugo hair. Kasabach–Merrit phenomenon, which is characterized by trapping of the platelets in the vasculature and thrombocytopenic coagulopathy leading to petechiae and ecchymosis is a known though uncommon complication of tufted angioma.

Histopathologically, tufted angioma is characterized by well-circumscribed foci of clustered capillaries in the dermis and the subcutaneous tissue. The capillaries are lined by plump or elongated endothelial cells. These ovoid tufts of capillaries give the “cannonball appearance” to the angiomatous structures. There is no mitotic activity or atypia in the cells.

It is important to differentiate tufted angioma from Kaposi's sarcoma, kaposiform hemangioendothelioma, and low-grade angiosarcoma, especially in adults. Kaposi's sarcoma shows spindle-shaped endothelial cells with slit-like spaces and the promontory sign. Kaposiform hemangioendothelioma shows a lobular and infiltrative pattern, whereas differentiation from low-grade angiosarcoma can be done by the absence of atypia and mitotic figures.

The various treatments that have been employed for tufted angioma include potent topical steroids, systemic steroids, interferon alpha, complete surgical excision, cryosurgery, radiotherapy, and pulsed dye laser.[3] However, the rate of recurrence is high.