ARTICLE
Auteur(s) : Alessia PACIFICO, Domenico PICCOLO, Maria
Concetta FARGNOLI, Ketty PERIS
Department of Dermatology, University of L'Aquila, Via
Vetoio Coppito 2, 67100 L'Aquila, Italy
Article accepted on 11/8/03
Kaposi's sarcoma (KS) is an angioproliferative disease of
spindle-shaped endothelial cells that has different clinical
variants, a particular geographical distribution and an
etiopathogenesis that is still unclear. Classic KS is an uncommon
form with a chronic course, that affects elderly men in the
Mediterranean region and Eastern Europe; endemic KS is more
aggressive and most common in equatorial Africa; iatrogenic KS
occurs in patients receiving immunosuppressive therapy; epidemic or
acquired immunodeficiency syndrome (AIDS)-associated KS is the most
aggressive form [1].
In recent years, detection of human herpes virus-8 (HHV-8) DNA
sequences in tissue specimens and blood in all forms of KS
suggested a major role of this virus as an infectious etiological
cofactor [2-4]. However, HHV-8 has been identified in benign
and malignant lymphoproliferative disorders such as body
cavity-based B-cell lymphomas and multicentric Castleman's disease
[5]. In addition, a large number of benign vascular lesions (i.e.
hemangiomas, lymphangiomas, pyogenic granulomas,
hemangiopericytomas, acroangiodermatitis and Kimura's disease) were
not found to contain the HHV-8 DNA sequences whereas HHV8 was
detected in angiolymphoid hyperplasia with eosinophilia;
controversial results have been reported in angiosarcoma [6-7].
We report the unusual localization of KS on the glans penis in a
39-year-old HIV-negative patient.
Case report
A 39-year-old Caucasian man presented with a solitary, slowly
growing, reddish-brown papule, located on the glans penis (Fig. 1). The papule
was soft and spongy to the touch and no other cutaneous lesions
were observed. The inguinal lymph nodes were not enlarged. Medical
history did not reveal HPV associated diseases nor
immunosuppressive therapy. Results of routine laboratory blood
tests, and serum immunoglobulin levels were within normal limits.
Blood tests were negative for human immunodeficiency virus (HIV)
1 and HIV 2 antibodies. The absolute number of
CD4+ T lymphocytes and the ratio of CD4+ to
CD8+ cells were normal. Gastrointestinal tract, liver
and lung examination revealed no evidence for systemic involvement.
Histopathologic examination of the papule showed an infiltrate
composed of spindle-shaped cells scattered between collagen bundles
and intermingled with small, pointed vascular-like spaces (Fig. 2).
Immunohistochemical investigations revealed positivity of the
spindle-shaped cells for anti-Factor VIII and
anti-CD34 antibodies and negativity for anti-CD31,
anti-S100 and anti-HMB-45 antibodies (Fig. 2). HHV-8 DNA
sequences were detected in tumor tissue and in peripheral blood
cells using a nested Polymerase Chain Reaction assay as described
previously [4]. Two months after the initial observation, three new
papules, ranging from 3 to 5 mm in diameter, developed on
the glans penis. Histopathologic examination of one lesion
confirmed the diagnosis of classic KS. The remaining lesions were
treated with electrodessication and curettage. After a follow-up
period of 6 months no evidence of recurrence was observed.
Discussion
Classic KS primarily affects males of Mediterranean or Eastern
European descent over the age of 60 years. Initial
manifestations usually consist of blue red patches and/or papules
that slowly progress to plaques or nodules and eventually ulcerate.
Preferential sites are the distal portion of the lower extremities.
In some cases, classic KS appears as a single lesion with no
tendency to progress or as a few lesions confined to a limited body
area. Occasionally, cutaneous lesions spontaneously regress
[8].
Isolated classic KS of the glans penis is rare with only
51 cases reported so far; among them, 38 cases have been
regarded as isolated classic KS without any association with AIDS
or immunosuppression [9-10]. In our patient, a solitary papular
lesion located on the glans penis represented the only clinical
manifestation in the absence of other cutaneous localizations as
well as systemic involvement for a 6-month period.
Clinical differential diagnosis included pyogenic granuloma,
molluscum contagiosum, condyloma acuminatum and bowenoid
papulosis.
Histopathologic findings in our patient were characteristic of KS.
In some cases however, differential diagnosis may include pyogenic
granuloma, angiosarcoma, hemangiopericytoma and fibrosarcoma.
The presence of HHV-8 in all forms of KS supports a causative
role for this virus in the development of KS [2-3]. In our patient,
HHV-8 could be detected in tumor tissue and peripheral blood.
Although most studies indicate that HHV-8 is spread by sexual
transmission, recently the presence of the virus in saliva of
infected individuals has been reported indicating a possible HHV8
transmission by this route [11-12].
Therapeutic approaches for KS include radiotherapy, cryotherapy,
surgical excision and intralesional injection of cytostatic
substances such as vinblastine or vincristine, as well as
intralesional or systemic interferon α [13-15]. Surgical excision
is the treatment of choice for patients presenting a solitary KS
lesion. We report this case for the unusual and unique localization
of classic KS on the glans penis in a young immunocompetent
patient. n
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