Autochthonous Cases of Mucosal Leishmaniasis in Northeastern Italy: Clinical Management and Novel Treatment Approaches

Abstract

Mucosal leishmaniasis (ML) is a rare clinical variant of tegumentary leishmaniasis in Mediterranean Europe. Here we report on three autochthonous cases of head and neck ML in patients living in Northeastern Italy. Patients presented with non-specific, long-standing symptoms of upper respiratory tract involvement, mimicking other diseases. Parasitological diagnosis was reached by histopathology, immunohistochemistry and molecular biology on tissue specimens. Leishmania infantum was identified by molecular typing in all three cases. All patients reached a complete remission with protracted multivalent antileishmanial drugs; in one case, a novel approach of combined medical and endoscopic surgical treatment was carried out. High clinical suspicion led to a prompt diagnosis and deployment of a multivalent treatment. ML should be considered in the differential diagnosis of nasal, oral, and pharyngolaryngeal lesions in endemic areas. A prompt diagnosis is mandatory to establish a correct management; different antileishmanial medications as well as endoscopic surgical options may be required to reach a complete remission.

Painful and swollen tongue: mucosal leishmaniasis due to Leishmania infantum

“…Almost all known cases of ML, including our own patient, were diagnosed by histological examination of a biopsy in which Leishmania amastigotes were found (Aliaga et al, 2003;Cobo et al, 2016;Cocuzza et al, 2013;Faucher et al, 2011;Franco et al, 2015;Gaspari et al, 2020;Guddo et al, 2005;Neumayr et al, 2012;Patel et al, 2017;Richter et al, 2011;Stoeckle et al, 2013). A positive PCR on the biopsy was found in most of the cases (Cobo et al, 2016;Cocuzza et al, 2013;Faucher et al, 2011;Gaspari et al, 2020;Guddo et al, 2005;Mohammadpour et al, 2017;Neumayr et al, 2012;Patel et al, 2017;Richter et al, 2011;Stoeckle et al, 2013), also in a small number of cases in which the histological examination was negative or not performed (Cobo et al, 2016;Faucher et al, 2011;Mohammadpour et al, 2017;Patel et al, 2017). In a part of cases, additional tests such as serology or Leishmania PCR on blood were performed (Aliaga et al, 2003;Cobo et al, 2016;Faucher et al, 2011;Franco et al, 2015;Gaspari et al, 2020;Guddo et al, 2005;Patel et al, 2017;Richter et al, 2011).…”

“…A positive PCR on the biopsy was found in most of the cases (Cobo et al, 2016;Cocuzza et al, 2013;Faucher et al, 2011;Gaspari et al, 2020;Guddo et al, 2005;Mohammadpour et al, 2017;Neumayr et al, 2012;Patel et al, 2017;Richter et al, 2011;Stoeckle et al, 2013), also in a small number of cases in which the histological examination was negative or not performed (Cobo et al, 2016;Faucher et al, 2011;Mohammadpour et al, 2017;Patel et al, 2017). In a part of cases, additional tests such as serology or Leishmania PCR on blood were performed (Aliaga et al, 2003;Cobo et al, 2016;Faucher et al, 2011;Franco et al, 2015;Gaspari et al, 2020;Guddo et al, 2005;Patel et al, 2017;Richter et al, 2011). Serology was positive in most of the patients (Aliaga et al, 2003;Cobo et al, 2016;Faucher et al, 2011;Franco et al, 2015;Guddo et al, 2005;Patel et al, 2017;Richter et al, 2011), while Leishmania PCR on blood was negative in the small number of tested patients (Faucher et al, 2011;Franco et al, 2015;Richter et al, 2011).…”

“…Our patient presented with ML due to L. infantum without prior history of VL or CL. Mucosal involvement in absence of concomitant visceral or cutaneous disease is rarely seen in L. infantum infections, but has been reported sporadically (Aliaga et al, 2003;Cobo et al, 2016;Cocuzza et al, 2013;Faucher et al, 2011;Franco et al, 2015;Gaspari et al, 2020;Guddo et al, 2005;Hammami-Ghorbel et al, 2015;Mohammadpour et al, 2017;Neumayr et al, 2012;Patel et al, 2017;Richter et al, 2011;Stoeckle et al, 2013). This is in contrast to ML due to L. brazilliensis, in which CL progresses to ML in a small part of the patients (Burza et al, 2018;World Health Organization, 2010).…”

“…ML due to L. brazilliensis starts typically on the nostrils or lips and can progress to deeper mucosa, which can cause severe mutilations if untreated (Burza et al, 2018;World Health Organization, 2010). In contrast, L. infantum, seems to favour other initial locations, such as the oral cavity (palatum, gingiva or tongue), intranasal mucosa, pharynx and larynx (Aliaga et al, 2003;Cobo et al, 2016;Cocuzza et al, 2013;Faucher et al, 2011;Franco et al, 2015;Gaspari et al, 2020;Guddo et al, 2005;Neumayr et al, 2012;Patel et al, 2017;Richter et al, 2011;Stoeckle et al, 2013). As most patients do not have a history of VL or CL, it remains the question if the lower parts of the upper respiratory tract (e.g.…”

“…In Europe, the most prevalent Leishmania species is L. infantum, which predominantly causes visceral leishmaniasis (VL) or cutaneous leishmaniasis (CL) (Burza et al, 2018;World Health Organization, 2010). However, localized ML in absence of concomitant VL or CL caused by L. infantum has been reported sporadically (Aliaga et al, 2003;Cobo et al, 2016;Cocuzza et al, 2013;Faucher et al, 2011;Franco et al, 2015;Gaspari et al, 2020;Guddo et al, 2005;Hammami-Ghorbel et al, 2015;Mohammadpour et al, 2017;Neumayr et al, 2012;Patel et al, 2017;Richter et al, 2011;Stoeckle et al, 2013). As the lesions mimic other diseases, the correct diagnosis is often delayed (Aliaga et al, 2003;Hammami-Ghorbel et al, 2015).…”