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Summary
Tufted angioma is an uncommon benign vascular neoplasm, localized to
the skin and subcutaneous tissues. Half of the cases present in the
first year of life. We describe a case in a 34-year-old male who
presented with different sized reddish nodules, occurring on
multiple hyperpigmented erythematous macules situated on the
proximal parts of upper limbs that first appeared three years
earlier. Histopathology revealed circumscribed multiple foci of
closely set vascular channels giving the canon ball appearance and
were of vascular origin .
Introduction
Tufted angioma is an uncommon benign vascular neoplasm, localized to the skin
and subcutaneous tissues with no documented systemic or metastatic involvement.
Tufted angioma were described in the literature under different names including
Nakagawa's angioma, Nakagawa's angioblastoma, progressive capillary hemangioma,
and acquired tufted angioma of the skin and subcutaneous tissue[1]. It is
characterized by slow
angiomatous proliferation with no racial predilection, and occurs equally in
both sexes. The skin lesions occur without a history of preceding trauma, except
for one case where tufted angioma has followed an arthropod bite[2].
The lesions are usually asymptomatic but painful episodes have been described [3]
.
Case Presentation
A 34-year-old male presented with multiple hyperpigmented erythematous
macules on the proximal parts of the upper limbs, shoulders, and dorsal aspects
of the forearms of 3-year duration. Nodules of different sizes appeared on top
of some of the macules. The nodular lesions varied in color from light to dark
red and were restricted to the macules, with no involvement of the normal skin.
The clinical differential diagnosis included Kaposi's sarcoma, pyogenic
granuloma, hemangioendothelioma, bacillary angiomatosis, and angiosarcoma.
Two biopsies were taken, one from the macular lesions, and the second from
one of the nodules. The first biopsy (from the macules) showed basal cell
hyperpigmentation, and dermal cellular infiltrate. The second biopsy (from one
of the nodules) showed multiple, discrete, circumscribed dermal foci of closely
set vascular channels, with the epidermis surrounding one of the foci in a
claw-like manner. The tumor masses appeared as dilated capillary channels lined
with spindle shaped infiltrating cells, with no atypia or mitotic figures, and
there were RBCs inside the cavities with no extravasations. All tumor cells
stained positively with CD34, while factor VIII was positive in some vessels.
CBC, prothrombin time, partial thromboblastin time, and
coagulation factors, and bleeding time were normal.
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| Figure 1 | Figure 2 |
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Reddish nodules, occurring on multiple hyperpigmented
erythematous macules.
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H&E section: tumor masses appeared as dilated capillary
channels lined with spindle shaped infiltrating cells, with no atypia or mitotic
figures, and there were RBCs inside the cavities with no extravasations.. |
Discussion
More than 50% of cases of tufted angioma occur within
the first year of life, and of those, 15% are believed to have had a blemish in
the area where tufted angioma appeared later, which suggests a congenital mode
of presentation[4]. Most of cases (60-70%) of tufted angioma develop before the
age of five. Fewer than 10% of cases with tufted angioma are older than 50
years. In individuals older than 60 years, the disease is very rare [5].
Tufted angioma presents clinically as ill-defined slowly progressive dull red,
brownish or purple macules or plaques with mottled surfaces. It slowly enlarges
over six months to ten years, after which no further growth occurs[6]. Papules and
nodules may superimpose on the main lesions[1] as was observed in the present
case.
The sites most commonly involved are the upper trunk, neck and shoulders, less
commonly the face, scalp, and proximal extremities. Rarely does it affect the
feet and oral mucosa. Partial regression may occur, but complete disappearance
is rare[7].
Hyperhidrosis may occur in the skin overlying the lesion, and when it occurs, it
corresponds histologically with an area of abundant collagen[4].
Occasional association of the more serious platelet-trapping syndrome (Kasabach-Merrit
syndrome) has been reported[8], so the presence of petechiae or ecchymotic patches
should alert the physician to the development of those complications. Cases of
tufted angioma have been reported on the skin of a liver allograft recipient,
but regressed after modulation of immunosuppressive therapy[9].
Different modalities of treatment have been tried, including complete surgical
excision, soft X-ray therapy, and electrocoagulation. Pulsed dye laser has also
been tried without encouraging results[10]. Potent topical steroids have been used
to reduce pain[2]. Interferon a2, and high doses of systemic steroids should be
tried before proceeding to excision, particularly where lesions are excessive[11, 12].
References
1. Alessi E, Bertani E, Sola F: Acquired tufted angioma. Am J Dermatopathol; 8: 426-9.
2. 2. Bernstein EF, Kantor G, Howe N, et al: Tufted angioma of the thigh. J Am Acad Dermatol 1994 31(2 Pt 2): 307-11.
3. Frenk E, Vion B, Merot Y et al: Tufted angioma. Dermatologica 1990; 181: 242-3.
4. 4. Ban M, Kaniya H, Kitajima Y: Tufted angioma of adult onset. Dermatology 2000; 201: 68-70.
5. Jang KA, Choio JH, Sung KJ et al: Congenital linear tufted angioma. Br J Dermatol 1998 138: 12-13.
6. Lam WY ,MacMoune-Lai F, Look CN et al: Tufted angioma with complete regression. Pathol 1994 21: 461-6.
7. Fukunaga M: Intravenous tufted angioma. APMIS 2000 ; 108: 287-92.
8. Enjolras O, Wassef M, Dosquet C, Drovet l et al: Kasabach-Merrit syndrome on a case of tufted angioma. Ann Dermatol Venereol 1998 ; 125: 257-60.
9. Chu P, Leboit PE:An eruptive vascular proliferation resembling acquired tufted angioma in the recipient of a liver transplant. J Am Acad Dermatol 1992; 26: 322-5.
10. Dewerdt S, Callens A, Machet L, Grangepont MC, et al: Acquired tufted angioma with failure of pulsed dye laser therapy. Ann Dermatol Venereol 1998 125: 47-93.
11. Park KC, Ahm PS, Lee YS et al: Treatment of angioblastoma with recombinant interferon α2. Pediatr Dermatol 1995; 12: 184-6.
12. Munn SE, Jackson JE, Fusseel-Jones R: Tufted haemangioma responding to high dose of systemic steroids: a case report and review of the literature. Clin Exp Dermatol 1994; 19: 511-14.
© 2005 Egyptian Dermatology Online Journal
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