ARTICLE
Auteur(s) : Franco
Rongioletti1, Giuseppe E Cannata2, Aurora
Parodi1
1Section of Dermatology, DISEM, University
of Genoa, Viale Benedetto XV, 7, 16132 Genova, Italy
2Section of Dermatology, Hospital of Imperia,
Italy
A 82-year-old, healthy, Italian man, who did not have any recent
history of travel outside Italy, was seen for a swelling of his
lips since 2 years. He had had a diagnosis of Miescher’s
macrocheilitis in another institution. Examination showed the
presence of an asymptomatic, severe swelling which involved the
lower lip and the left side of the upper lip with the presence of
crusts and scaling and a commissural cheilitis (figure 1A). Regional
lymphadenopathy was absent. Histolopathology showed a dense dermal
inflammatory cell infiltrate composed of lymphocytes, histiocytes
and plasma cells. High power magnification revealed the presence of
abundant intra- and extra-cellular Leishmania amastigotes (figure 1B). Culture
on Novy-MacNeal-Nicolle medium was positive for Leishmania spp.
Polymerase chain reaction was positive for Leishmania infantum.
Other laboratory and instrumental examinations excluded a visceral
localization. The patient did not tolerate IL N-methylglucamine
antimonate for pain and was treated with amphotericin B in its
liposomal form (3 mg/kg/d on days 1-5) with complete
resolution of the macrocheilitis in 4 weeks (figure 1C). During
follow-up of one year, no relapses were observed.
Discussion
Cutaneous leishmaniasis (CL) is characterised by a spectrum of
clinical manifestations going from a solitary ulcerative lesion
developing at the site of the sandfly bite (localised cutaneous
leishmaniasis [LCL]) to multiple non-ulcerative nodules (diffuse
cutaneous leishmaniasis [DCL]) to destructive mucosal inflammation
(mucosal leishmaniasis) and disseminated visceral leishmaniasis. In
the Old World, L. tropica, L. major and L. killicki are usually
responsible for LCL, while L. aethiopica can cause both LCL and
DCL, and L. infantum and L. donovani can cause both LCL and
visceral leishmaniasis. However, in addition to the various
species, the different clinical presentations of CL depend on the
site of bite, the type of parasite and host factors [1].
In the Mediterranean basin, including Italy, where CL is very
frequent, the typical presentation is a solitary lesion on the face
(the so-called “Oriental sore”). CL located on the lips and
presenting as macrocheilitis is a difficult clinical challenge.
Although rarely reported in the literature, leishmaniasis of the
lip is not uncommon in Saudi Arabia or Turkey, occurring mainly in
young people [2]. In the rare cases in which it was possible to
determine the responsible species, L. donovani, L. infantum, L.
major, and L. tropica have been demonstrated. On the contrary, lip
involvement in Italy has been described in only four cases [3-6],
including an immunosuppressed renal transplant recipient [4] and a
patient with Down’s syndrome [5]. The rarity of such a presentation
suggests that not only are the lips an uncommon site of inoculation
but also some predisposing factors and immune dysfunctions can play
a role [5]. In all reported cases, L. infantum has been
demonstrated, as in our case. However, no predisposing factors or
immunological abnormalities were found in our patient.
The most important differential diagnoses include bacterial
infections, syphilitic chancre, granulomatous cheilitis, and
squamous cell carcinoma. Histopathology is crucial for the right
diagnosis. Mucocutaneous leishmaniasis caused by L. brasiliensis,
L. guyanensis and L. panamensis are ruled out both on epidemiology
and clinical grounds with the absence of destruction and serious
disfiguration and on the identification of the specific strain.
All of patients with leishmaniasis of the lip were successfully
treated with pentavalent antimony derivatives, by either IV, IM, or
intralesional route [6], with the exception of a case of
spontaneous complete remission. In our case, the unbearable pain
with IL meglumine antimonite and the identification of L. infantum
as causative agent, with its potentiality to cause visceral
involvement, prompted us to start systemic treatment with
amphotericin B in its liposomal form with complete healing of the
macrocheilitis in a month.
Acknowledgements
Conflict of interest: none. Financial support: none.
References
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Surg Oral Med Oral Pathol Oral Radiol Endod 2007; 104: 659-61.
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