ARTICLE
A healthy 30-year-old woman consulted us for a solitary skin lesion located
on the right shoulder which had been present for one year.
Physical examination showed a raised purple papule, 1 cm in diameter,
surrounded by a thin, pale area and a peripheral ecchymotic ring (Fig.
1).
The patient reported that the lesion occasionally became swollen, tender
and then, the ecchymotic ring spontaneously disappeared, resembling an
hematoma in various stages of resolution.
HIV serology was negative.
Targetoid hemosiderotic
hemangioma
Histological examination was consistent with the diagnosis of targetoid
hemosiderotic hemangioma, showing a poorly circumscribed vascular proliferation
with a superficial and a deep component, resulting in a biphasic growth
pattern.
In the papillary dermis (Fig.
2), vascular channels were irregular and dilated, and lined by
a single layer of epithelioid endothelial cells. Some papillary projections
were noted.
In the deeper dermis and the hypodermis (Fig.
3), vascular structures were thinner, angulated and more infiltrative,
dissecting the collagen bundles, extending along skin appendages and taking
on an angiomatous aspect. Important lymphocytic inflammation was present
and some extravasated red blood cells were seen. No hemosiderin deposits
were seen.
Endothelial cells, characterized by a typical "hobnail" appearance,
were noted in the whole proliferation, without mitoses or cytological
atypia (Fig. 4).
Immunohistochemical study revealed a positive staining with the following
antibodies: factor VIII, CD31, BNH 9. The pattern of staining was similar
for the superficial and deep components. No staining was observed for
CD34. This negativity, which has been confirmed, is surprising in such
a benign vascular proliferation.
Comments
Targetoid hemosiderotic hemangioma (THH) is a rare benign vascular tumor
first described by Santa Cruz and Aronberg in 1988 [1].
Age at presentation is variable (from 7 to 55 years) and the lesion
can be located anywhere [1, 2]. Acute onset is usual [1] and it has been
noticed after pregnancy in two cases [3, 4].
The characteristic clinical presentation is that of a small, solitary,
violaceous papulo-nodule, smaller than 1 cm in diameter, surrounded by
successive flat, clear and ecchymotic rings giving a typical targetoid
appearance.
However, this targetoid aspect may fluctuate
and, as in our patient, the ecchymotic halo may disappear after having
been swollen and tender.
The targetoid appearance is not absolutely specific since it has also
been reported in cherry angiomas in patients with primary amyloidosis
or in Lyme disease [1, 3].
The histological hallmarks of THH include a biphasic architecture (dilated
vascular channels in the superficial dermis, angulated and infiltrative
vascular proliferation in the deep dermis and the hypodermis) and endothelial
cells with a "hobnail" appearance [1, 5]. There is no cytological atypia.
Lymphocytic infiltrate is usual and may occasionally be very prominent
[6].
Hemosiderin deposits are inconsistent [4, 7] and we believe that their
absence in our case does not rule out the diagnosis. A better denomination
for this lesion may be "hobnail hemangioma" [7] or "targetoid hemangioma"
[4].
Histological differential diagnoses of THH include
patch stage Kaposi's sarcoma and other vascular tumors with "hobnailed"
endothelial cells.
The presence of spindle-shaped endothelial cells with intracytoplasmic
eosinophilic bodies and lymphocytic and plasmocytic infiltrate favors
Kaposi's sarcoma [5-7]. Additional, nontargetoid lesions and HIV seropositivity
are important clinical and biological data for differential diagnosis.
Distinction between malignant endovascular papillary angioendothelioma
(Dabska's tumor) and retiform hemangioendothelioma can be difficult. However,
the latter is clinically different and a distinctive morphological feature
is the presence of long, arborizing blood vessels resulting in a retiform
pattern, like normal rete testis [7].
Finally, the distinction between well-differentiated angiosarcoma can
be difficult but this tumor occurs most often on the head and the neck
of elderly patients and histologically, there is a much more disorganized
pattern and cytological atypia [7].
REFERENCES
1.Santa Cruz DJ, Aromberg J. Targetoid hemosiderotic hemangioma. J
Am Acad Dermatol 1988; 19: 550-8.
2. Vion B, Frenk E. Targetoid hemosiderotic hemangioma. Dermatology
1992; 184: 300-2.
3. Rapini RP, Golitz LE. Targetoid hemosiderotic hemangioma. J Cutan
Pathol 1990; 17: 233-5.
4. Morganroth GS, Tigelaar RE, Longley J, Luck LE, Leffell DJ. Targetoid
hemangioma associated with pregnancy and the menstrual cycle. J Am
Acad Dermatol 1995; 32: 282-4.
5. Lowe L. Targetoid hemosiderotic hemangioma. Self-assessment. J
Cutan Pathol 1994; 21: 567-9.
6. Perrin C, Rodot S, Ortonne JP, Michiels JF. L'hémangiome en
cible hémosidérinique. Ann Dermatol Vénéréol
1995; 122: 111-4.
7. Calonje E, Fletcher CDM, Wilson-Jones E, Rosai J. Retiform hemangioendothelioma.
Am J Surg Pathol 1994; 18 (2): 115-25.
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