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Year : 2018  |  Volume : 19  |  Issue : 2  |  Page : 182-183

Tufted angioma


1 Department of Dermatology, R.N.T. Medical College, Udaipur, Rajasthan, India
2 Department of Venereology and Leprology, R.N.T. Medical College, Udaipur, Rajasthan, India

Date of Web Publication26-Mar-2018

Correspondence Address:
Sharad Mehta
Department of Dermatology, R.N.T. Medical College, Udaipur - 313 001, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_42_17

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How to cite this article:
Mehta S, Meena M, Goyal U, Gupta LK, Khare AK, Mittal A. Tufted angioma. Indian J Paediatr Dermatol 2018;19:182-3

How to cite this URL:
Mehta S, Meena M, Goyal U, Gupta LK, Khare AK, Mittal A. Tufted angioma. Indian J Paediatr Dermatol [serial online] 2018 [cited 2019 Dec 7];19:182-3. Available from: http://www.ijpd.in/text.asp?2018/19/2/182/211817



Sir,

A 7-year-old girl child presented with 2½ year history of gradually progressive asymptomatic, plaques affecting face, neck, and upper back.

Examination revealed multiple, red to violaceous, well defined, smooth, soft, nontender, infiltrating plaques of variable shape and size ranging from 2 cm × 3 cm to 5 cm × 6 cm. Prominent hypertrichosis was seen in all lesions [Figure 1]. Lesions over the mandibular lesion showed nodularity. Nail, hair, mucosa, general physical, and systemic examination were with in normal limits. The child was in good general health. Hemogram and serum biochemistry was normal.
Figure 1: Red to violaceous, well defined, smooth, infiltrating plaques with prominent hypertrichosis affecting face, neck

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Histopathologic examination revealed lobules of tightly packed, proliferating blood filled capillaries with scattered inflammatory cells in intervening stroma [Figure 2]. There was no evidence of granuloma. Lymphohistiocytic aggregates were seen around neurovascular bundles along with dermal collagenization. Periodic acid-Schiff and Ziehl–Neelsen staining were negative. The histopathology was consistent with the diagnosis of tufted angioma (TA).
Figure 2: Lobules of tightly packed, proliferating blood filled capillaries with scattered inflammatory cells in intervening stroma in dermis (H and E, ×10)

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Tufted angiomas are rare benign vascular tumors of unknown pathogenesis, which typically occur in infancy or early childhood.[1] These classically present as slowly expanding, mottled, red-to-purple patches, and firm plaques with superimposed papules and nodules, mainly on the neck, shoulders, trunk, or groin. The lesions are often associated with pain or tenderness, localized hyperhidrosis, and hypertrichosis.[2],[3]

About 15% of cases are congenital, occasionally associated with Kasabach–Merritt syndrome (KMS), a thrombocytopenic coagulopathy caused by platelet trapping within lesional vessels.[4] Approximately, 10% of cases show spontaneous regression over a period of 6 months to 2 years.[5] The literature does not suggest it is aggressive behavior or metastatic tendency.

Histopathologically, TA is characterized by multiple, discrete lobules of tightly packed capillaries in a cannonball pattern within the dermis and sometimes extending up to subcutis.

TA can closely simulate Kaposiform hemangioendothelioma (KHE), and their differentiation may be challenging. Some authors consider both to be within the spectrum of the same condition. TA tends to be located more superficially compared to KHE which may affect subcutis and retroperitoneum, invade the internal organs and is characterized by aggressive growth. Angiosarcomas usually affect elderly patients and have a predilection for the scalp. Multiple mitotic figures and marked atypia are the hallmarks of angiosarcomas.[3]

TA should be closely monitored due to its propensity to cause KMS, especially in early childhood. In uncomplicated cases “active nonintervention” with close surveillance is a reasonable approach, as spontaneous regression can occur. TAs that are complicated by KMS, rapid growth, or functional compromise, for example, airway obstruction or joint contracture, have been treated with systemic glucocorticoids, vincristine, and interferon-α with variable efficacy.[3] Surgical excision is an option for small lesions, but recurrences are common. Our patient did not show any evidence of KMS and is under close surveillance without any active intervention.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
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(2 Pt 1):214-25.  Back to cited text no. 1
    
2.
Wong SN, Tay YK. Tufted angioma: A report of five cases. Pediatr Dermatol 2002;19:388-93.  Back to cited text no. 2
[PUBMED]    
3.
Herron MD, Coffin CM, Vanderhooft SL. Tufted angiomas: Variability of the clinical morphology. Pediatr Dermatol 2002;19:394-401.  Back to cited text no. 3
[PUBMED]    
4.
Arai E, Kuramochi A, Tsuchida T, Tsuneyoshi M, Kage M, Fukunaga M, et al. Usefulness of D2-40 immunohistochemistry for differentiation between kaposiform hemangioendothelioma and tufted angioma. J Cutan Pathol 2006;33:492-7.  Back to cited text no. 4
[PUBMED]    
5.
Ishikawa K, Hatano Y, Ichikawa H, Hashimoto H, Fujiwara S. 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;210:346-8.  Back to cited text no. 5
[PUBMED]    


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