Nahed. Ateyya *, M. F Botros*, I. Abdo*, S. Foda*, H. Amer*, , S. Saad*,
G. Foda*, M. Esmat*, , M.El-Darouti** and M. A. Abdallah***
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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 .
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. 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.
The lesions are usually asymptomatic but painful episodes have been described 
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.
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. 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 .
Hyperhidrosis may occur in the skin overlying the lesion, and when it occurs, it
corresponds histologically with an area of abundant collagen.
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. Potent topical steroids have been used to reduce pain. Interferon a2, and high doses of systemic steroids should be tried before proceeding to excision, particularly where lesions are excessive[11, 12].
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