Department of Dermatology, New York University
A 4-month-old girl developed coalescing, red-purple, firm plaques with irregular borders and superimposed papules in the left groin. The lesions were tender to palpation and they slowly expanded and became thicker over the next year. Histopathologic evaluation showed multiple, discrete lobules of tightly packed capillaries in a 'cannonball' pattern within the dermis, which confirmed the diagnosis of tufted angioma. The clinical and histopathologic features, natural history, and treatment options for tufted angiomas are reviewed; their relationship to kaposiform hemangioendotheliomas is discussed.
The patient presented to the Dermatologic Associates at New York University Medical Center in August, 2005, with a two-month history of red plaques in the diaper area. The lesions had developed at age four months and were painful when wiped during diaper changes. The plaques slowly expanded and became thicker over the next year and then stabilized. She was otherwise healthy and her growth and development were normal. There was no family history of similar vascular lesions.
Coalescing, red-purple, firm plaques with irregular borders and a cobblestone-like surface due to superimposed papules were present in the left groin. The area of involvement extended to the lower abdomen and the superior part of the anterior thigh. The lesions were mildly tender to palpation.
A complete blood count was normal.
Throughout the dermis, there are lobules composed of small, tightly packed capillaries, some of which bulge into dilated, thin-walled vascular structures.
Tufted angioma is an uncommon, benign, vascular tumor that usually develops during infancy or childhood, with the majority of lesions arising before the age of five years . It typically presents on the neck, shoulders, trunk, or groin as slowly expanding, mottled, red-to-purple patches and firm plaques with superimposed papules and nodules. Lesions are often associated with paroxysmal pain or tenderness to palpation and localized hyperhidrosis and hypertrichosis have been observed . There are rare reports of a familial occurrence of tufted angiomas.
Histopathologically, tufted angiomas are characterized by multiple, discrete lobules of tightly packed capillaries in a cannonball pattern within the dermis and sometimes subcutis. Tufted angiomas, especially the congenital subset that accounts for approximately 15 percent of cases, are occasionally associated with Kasabach-Merritt syndrome (KMS), which is a thrombocytopenic coagulopathy that is caused by platelet trapping within lesional vessels. Tufted angiomas are thought to exist on a spectrum with kaposiform hemangioendotheliomas (KHEs), which are vascular tumors that are deeper, locally aggressive, and more frequently associated with KMS. In tufted angiomas, the capillary lobules (which do not stain with lymphatic markers) arise within a field of ectatic lymphatic vessels; in contrast, the fascicles of spindle cells that compose KHEs stain with lymphatic markers .
Spontaneous regression usually occurs over a period of six months to two years in approximately 10 percent of tufted angiomas and is most common in lesions with onset before the age of six months [4, 5]. Central involution and peripheral expansion may result in an annular appearance. Tufted angiomas that are complicated by KMS, rapid growth, or functional compromise (e.g., airway obstruction or joint contracture) have been treated with systemic glucocorticoids, vincristine, and interferon-α with variable efficacy . Surgical excision is an option for small lesions, but recurrences are common. Treatment with superpotent topical glucocorticoids, the pulsed dye laser, or intense pulsed light may alleviate pain associated with tufted angiomas .
References1. Jones EW, Orkin M. Tufted angioma (angioblastoma): a benign progressive angioma, not to be confused with Kaposi's sarcoma or low-grade angiosarcoma. J Am Acad Dermatol 1989; 20: 214 PubMed
2. Herron MD, et al. Tufted angiomas: variability of the clinical morphology. Pediatr Dermatol 2002; 19: 394 PubMed
3. Arai E, et al. Usefulness of D2-40 immunohistochemistry for differentiation between kaposiform hemangioendothelioma and tufted angioma. J Cutan Pathol 2006; 33: 492 PubMed
4. Ishikawa K, et al. The spontaneous regression of tufted angioma: a case of regression after two recurrences and a review of 27 cases reported in the literature. Dermatology 2005; 210: 346 PubMed
5. Browning J, et al. Congenital, self-regressing tufted angioma. Arch Dermatol 2006; 142: 749 PubMed
6. Mahendran R, et al. Response of childhood tufted angioma to the pulsed-dye laser. J Am Acad Dermatol. 2002; 47: 620 PubMed
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