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The First Probable Case of Leprosy in Southeast Italy (13th-14th Centuries AD, Montecorvino, Puglia)

The First Probable Case of Leprosy in Southeast Italy (13th-14th Centuries AD, Montecorvino, Puglia) Hindawi Publishing Corporation Journal of Anthropology Volume 2012, Article ID 262790, 7 pages doi:10.1155/2012/262790 Research Article The First Probable Case of Leprosy in Southeast Italy (13th-14th Centuries AD, Montecorvino, Puglia) 1, 2 2 2 2 1 Mauro Rubini, Valentina Dell’Anno, Roberta Giuliani, Pasquale Favia, and Paola Zaio Anthropological Service, SBAL, Via Pompeo Magno 2, 00193 Rome, Italy Department of Archaeology, Foggia University, Piazza Civitella 2, 71121 Foggia, Italy Correspondence should be addressed to Mauro Rubini, antropologiasal@libero.it Received 21 March 2012; Revised 3 June 2012; Accepted 25 June 2012 Academic Editor: Scott M. Fitzpatrick Copyright © 2012 Mauro Rubini et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. In 2008, during an archaeological excavation on the medieval site of Montecorvino (Foggia, Puglia, Italy), ten individuals were found buried near the principal church. The tombs were dated to the 13th-14th centuries AD, except for one attributable to the 11th century AD. The individual from tomb MCV2 shows some bone changes in the rhinomaxillary area. The most probable diagnosis is that she suffered from a type of near-multibacillary leprosy. Although leprosy has been documented in Italy from the first millennium BC and well described in the first millennium AD, its presence seems to be confined to Northern and Central Italy. This is the first case of leprosy in southeastern Italy and the second in Southern Italy overall. At the moment, the interesting datum is that leprosy seems to appear in Southern Italy only after the first millennium AD. All this could be because of the First Crusade with the opening of new trade and pilgrimage routes to the Near East or simply because other cases of leprosy have still not been found in osteoarchaeological context. 1. Introduction over 60 [6, 7]. Response to the disease is highly variable, and the immune status of each infected person determines Leprosy, or Hansen’s disease, is a chronic infection caused the type and severity of pathological change [8]. In its most by an unculturable pathogen, Mycobacterium leprae.It severe form, multibacillary (MeSh-lepromatous) leprosy, affects the peripheral nervous system primarily, especially both the skin lesions and the peripheral nerve damage the extremities (neural leprosy), and secondarily involves become generalised and symmetrical in nature. Those with the skin, nasal tissues, and bones [1]. As is well known, high immune status are more likely to develop paucibacillary Mycobacterium leprae is a highly infective bacterium with low (MeSh-tuberculoid) leprosy, where only a small number pathogenicity; that is, its ability to induce clinical disease is of skin lesions develop and peripheral nerve damage is low [2]. The bacteria affect the motor, sensory, and auto- asymmetrical. Having reached endemic status in mediaeval nomic nervous systems. M. leprae accumulates principally Europe, leprosy declined in the postmediaeval period about in the extremities of the body, where it resides within the the 14th century AD [9–11]. The decline of leprosy in macrophages and infects the Schwann cells of the peripheral Western Europe implied a complex web of factors, especially nervous system. Lack of myelin production by infected medical, environmental, social, and legal [12]. Under the Schwann cells and their destruction by host-mediated biomedical aspect, this decline probably may have been immune reactions lead to nerve damage, sensory loss, and caused by the cross-infection between leprosy and tuberculo- the disfiguration that, sadly, are the hallmarks of leprosy [3]. sis [13–15], although this possibility is debate still today and, Primary transmission occurs through droplet infection [4]. the exact relationship between TB and leprosy still remains The incubation period is very long (in some cases 20 years) unclear [16]. before clinical signs and symptoms become apparent [5]. In Italy, the skeletal documentation on leprosy is scarce. Leprosy can affect all age groups and seem to prefer male sex, According to the osteoarchaeological evidence, it was present although it is often first observed before the age of 20 and in the Northeast in the first millennium BC [17], in Central 2 Journal of Anthropology Figure 1: Geographic location of the site. Italy during the Roman period near Rome with the cases of Palombara [18] and Martellona [19] and in the early Middle Ages with the cases of Campochiaro Vicenne [20]and Campochiaro Morrione [21]. Furthermore, there is another late medieval case in South Italy [22]. Leprosy is rarely found today in Europe but is still a significant disease worldwide, principally in Southeast Asia, Africa, and South America (Brazil) [23]. The aim of this study is to investigate and discuss the skeleton of an individual from the medieval church of Montecorvino that shows interesting rhinomaxillary changes. Figure 2: Tomb MCV2. 2. Material and Methods In 2008, during an archaeological excavation in the medi- on the skeleton was effected according to the suggestions aeval town of Montecorvino (Foggia, Puglia—Figure 1), ten of Andersen and Manchester [32], Roberts and Manchester skeletons were found buried in graves near the principal [33], and Nunzi, and Massone [5]. church. The chronology obtained from the gravegoods is 13th-14th century AD, except for one dated to the 11th 3. Description of the Skeleton century AD. The skeleton under study was found in a tomb that was built with bricks near the perimetral wall of the MCV2 is a skeleton attributable to a female aged 35–40 years. church (Figure 2) and was found without covering. The The examination of the skeletal remains shows some bone code number of this individual is MCV2. The only object changes in the rhinomaxillary region. The alveolar process of found with it is an iron belt-buckle, which was dated by its the maxilla shows an intense erosive activity in the anterior particular shape to the end of the 13th century AD (Favia, side with loss of bony tissue producing the exposure of the personal communication). The skeletal remains are quite cavity of the root of the left and right central incisors and well preserved but unfortunately incomplete (Figure 3). The right lateral incisors, which are all absent (see Figure 4(a)). diagnosis of sex and age at death was carried out according The loss of teeth was surely antemortem because the alveolar to the standard suggested by Buikstra and Ubelaker [24]. In cavities show a discrete atrophy. In the left side, a damage particular, sex determined by the morphology of the os coxae postmortem is present in the alveolar portion of the lateral [25–27]and cranium[28]. Age at death was calculated on the incisor and canine. Although the area is damaged in the morphological changes of the pubic symphyseal face [29], on left side, the anterior nasal spine could be resorbed (see the auricular surface of the os coxae [30] and on the cranial Figure 5(a)) because of visible traces of little bone neoforma- suture closure [31]. The screening of the traces of leprosy tion. There is a symmetrical resorption and remodelling of Journal of Anthropology 3 (a) Figure 3: Bones present (grey) in individual MCV2. the normally sharply defined inferior margins of the piriform aperture with irregular rounding of the external profile (see Figure 5(a)), while the internal nasal surface is pitted (Figure 4(b)). The right lateral margin is smooth and thick in (b) appearance, while the left is damaged. Furthermore, the left and right inferior nasal conchae were partially resorbed. In Figure 4: (a) Alveolar process of the maxilla with an intense erosive conjunction with the remodelling of the margins of the nasal process in anterior part and loss of bony tissue with exposure of the aperture, the alterations of the intranasal structures (that are tooth roots in the right side and of the left central incisor (black not damaged) seem in the appearance of a wide, semiempty arrows). Furthermore, the margins of the piriform aperture are cavity (see Figure 5(a)). The alveoli of the anterior teeth rounded (white arrows). (b) Particular of the nasal cavity. On the shows an irregular shape in the lingual edge, and in the hard bony wall there is presence of pitting (arrows). palate there is an intense presence of pitting that involves also the alveolar cavity of the right and left molars (Figure 5(b)). In the postcranial skeleton only a light periostitis is present infections caused by spirochetes of the genus Treponema. on the tibiae in mesiodistal position. Excluding pinta, which does not affect bones,in the tertiary stage these diseases can involves the skeleton, and they tend 4. Differential Diagnosis to be associated with inflammatory bony changes accompa- There are various pathological conditions that could lead nied by extensive bone regeneration, often resulting in alter- to the destruction of bone in the rhinomaxillary region ation of the bone morphology and in some cases destruction [34]. These disorders include granulomatous diseases such of the nasal-palatal area may occur [34]. Yaws is typical of the as sarcoidosis and treponemal diseases and fungal infections humid tropical regions of South America, Africa, Asia, and such as aspergillosis and mucormycosis (phycomycossis), acti- Oceania. In this disorder, the last stage can be characterized nomycosis (a bacterial rather than a true fungal disease), and by widespread bone, joint, and soft tissue destruction, which lupus vulgaris (tuberculosis of the facial skin and soft tissue). may include extensive destruction of the bone and cartilage Sarcoidosis is a granulomatous disease of unknown aeti- of the nose (rhinopharyngitis mutilans or “gangosa”). Joints ology. Like leprosy, it tends to affect the phalanges of the may stiffen, and chronic osteitis and periostitis can lead to fingers and toes, causing lytic lesions and no reactive bone deformed leg bones (sabre tibiae). Bejel (endemic syphilis) formation [34]. However, in the skull, it causes mainly is present in nomadic populations of the Sahel and more in the destruction of the nasal bones while only rarely of the general in the desert region of Africa, in the Middle East, anterior nasal spine and never of the crest [35]. Thus, we can Central Australia, and Asia. Periostitis of the leg bones is exclude this disease as an explanation for the bony lesions in commonly seen. The involvement of the skull is very rare, our specimen. In view of the reconsideration of the origin with gummas of the soft palate and nose developing in the of treponemal disease in the Old World, we may also discuss last stage [35]. The “gangosa” condition may occur rarely. this group of pathologies since, like leprosy, they can lead to In venereal syphilis, the most commonly affected bones are the destruction of the nose and to bony changes in the lower (in order of importance) tibia, frontal and parietal (with limbs [34]. Treponemal diseases include syphilis (venereal caries sicca on the outer tables), nasal-palatal region, clavicle, and endemic), yaws and pinta, which are granulomatous sternum, vertebrae, fibula, femur, humerus and radius, and 4 Journal of Anthropology Mucormycosis (phycomicosysis) is a rare invasive fungal infection, which tends to affect people who have poorly controlled diabetes. It results in black, dead tissue in the nasal cavity and blocks in the blood supply to the brain, leading to neurological symptoms, such as headaches and blindness [38]. Mucormycosis attacks the nasal cavity with involvement of the paranasal sinuses and their walls [34]. A diagnosis of aspergillosis and mucormycosis can be ruled out, because in no case is the unilateral and bilateral perforation of the hard palate present. Furthermore, no traces of this pathology are present in the orbit region of the Montecorvino skeleton. Actinomycosis produces effects on the cervicofacial area; bone involvement is rare. When affected, the mandible rather than the maxilla is more involved [34, 39]. This is not the case with our individual. (a) Lupus vulgaris is a chronic tubercular infection of the skin involving soft yellow swellings, ulcers, and abscesses. Long-standing tuberculosis of the facial skin and soft tissues can lead to the destruction of the nasal bones [35]. The anterior alveolar process, however, is rarely affected [37], which discounts lupus vulgaris as a diagnosis. Given the localization of the lesions in our sample, the most likely diagnosis is multibacillary leprosy [32, 33, 37, 40]. Some bone changes are present in the rhinomaxillary region. The enlargement and rounding of the piriform aperture, the cicatrisation of the lower margin of the inferior nasal aperture, and erosion of the alveolar margin accompanied by the loss of the front teeth could be changes seen in leprosy referred to as rhinomaxillary syndrome [32, page 122]. Although the area is damaged, also the resorption of the ante- rior nasal spine could be present. Furthermore, the presence of pitting shows a presence of a chronic inflammation in the (b) nasal-palatal region. Deformity of the nasal-maxillary facial Figure 5: (a) Particular of the possible resorption of the anterior structures results from primary skeletal infection by Myco- nasal spine with bone neoformation (black arrows) in the right part bacterium leprae [34, 41], and alterations of the hands and (the left is partially damaged) and remodelling of the margins of the feet (especially the fifth metatarsal bone, where the same nasal aperture with presence of bony neoformation (white arrows). nerves that enervate the fibula are present) are secondary (b) Hard palate. On the surface an intense pitting process is present to pathological changes in the peripheral nerves with conse- that involve also the alveolar cavity of the left and right molars. quent loss of sensory and motor functions [42]. These last osteological markers are not present in our sample, but a light periostitis in the lower limbs is present also if aspecific. ulna. The teeth may also be involved, showing a screw- Periostitis, also known as periostalgia, is a clinical condition driver shape (Hutchinson teeth). No specific involvement of caused by inflammation of the periosteum, a layer of con- the feet is observed in this pathology [34, 35]. The most nective tissue that surrounds bone. The condition is generally common bones afflicted during the tertiary stage of the chronic and is marked by tenderness and swelling of the bone and an aching pain [35]. It could be due to scurvy, fractures, disease are the tibia (with sabre-shaped deformity) and the skull. According to Ortner [34], the calvarial lesions are the and infectious diseases such as, for example, treponematosis most specific diagnostic features. Although the nasal cavity and leprosy [35]. In this case, in absence of fractures or scurvy it is probably secondary and associated with an is often enlarged, producing the characteristic “saddle nose,” the nasal spine is usually spared [36]. Furthermore, the infectious disease. anterior alveolar change process is uncommon in yaws and syphilis [37, page 297]. Therefore, the pathogenetic picture 5. Discussion and Conclusions of our skeleton does not correspond to that of treponemal diseases. The study of the pathological changes of the skeleton from Aspergillosis is marked by inflammatory granulomatous the church at Montecorvino shows that this individual lesions in the skin, ear, orbit, nasal sinuses, lungs, and probably suffered from a type of near multibacillary leprosy. sometimes bones and meninges. It affects the paranasal When leprosy affects the skeleton, a number of specific and sinuses and orbit or the anterior cranial fossa [35]. nonspecific bony and osteoporotic changes occur during Journal of Anthropology 5 The route of the First Crusade (1096–1099) Kilometers Figure 6: The route of the First Crusade (1096–1099). pathogenesis [43]. Specific bony changes are caused by inva- publication of leprosy cases in Italy has increased (for a sion of the tissues by M. leprae, which is why facial changes survey see [21]). During the first millennium AD, there are are seen; secondary bone changes are a result of peripheral no osteological cases found in southern Italy, although many nerve involvement (hands, feet and lower leg bones) and lead prehistoric and historic skeletal collections have been studied to sensory nerve anaesthesia, trauma to the hands and feet by anthropologists and palaeopathologists. At present, the because people cannot feel the trauma, then ulceration of the most ancient cases known are that of Henry VII in Calabria feet and hands, then secondary infection of the bones of the (southwest Italy) dated to the thirteenth century AD [22] hands and feet [5]. According to Nunzi and Massone [5], the and now that of Montecorvino (southeast Italy) dated major skeletal foci of leprosy are the face and small tubular to the thirteenth-fourteenth century AD. The spread of bones of the hands and feet. Rarely also other bones may be leprosy in Southern Italy could be subsequent to the first affected [34]. The mediaeval individual from Montecorvino wave that afflicted Northern and Central Italy during the shows some traces of leprosy, with a possible rhinomaxillary first millennium AD [21]. In fact, during this period, the syndrome. Although pathogenesis of the skeletal changes in phenomenon of the “Migration of the Peoples” or barbarian leprosy is generally difficult to assess in osteoarchaeological invasions produced an increase in epidemic diseases in Italy remains, Ortner [34] observed that the presence of destruc- [18]. An explanation for the presence of leprosy only in tive and proliferative lesions could indicate the infectious Northern and Central Italy in the first millennium AD could phase (chronic or acute) of the disease, without specifying be found in the pattern of Monot et al. [46] based on the use the pathological process that caused the bone changes. In of comparative genomics. Monot et al. demonstrated that the fact, Mycobacterium leprae prefers cool environments, and single clone responsible for leprosy can be traced by analysis the distribution of the lesions in multibacillary leprosy is of rare single-nucleotide polymorphisms (SNPs). They have associated with the cooler, exposed areas of the skin and obtained four permutations (types) of SNPs distributed mucous membranes like that within or under the nose or throughout the world. According to these results, leprosy more generally of the face, which constitutes the elective seem to have originated in eastern Africa or the Near East site of aggression of the disease [1]. Leprosy does not cause and spread with successive human migrations. According to death; rather, people usually die of TB [13, 14]. Therefore, the the scheme of Monot et al., from about 40,000 years BP different manifestations of the disease in osteoarchaeological the disease’s path was the same as that of the route of the remains are not homogeneous but can show numerous human populations obtained from genetic and anthropo- variants [34]. Furthermore, the last consideration is that logical data and was directed from Central Asia/Eastern MCV2 was buried in the normal cemeterial area of the Europe to Western Europe [47]. This human flow would church. This is not infrequent during this period. Usually seem to be uninterrupted during the first millennium AD individuals with leprosy were buried in normal cemetery preferring terrestrial way [21]. This last observation could [9, 20, 21, 44, 45]. be confirmed by the geographical location of the Italian The mediaeval individual from Montecorvino is the first osteoarchaeological evidences. case of leprosy described in the literature in southeastern After the first millennium AD, probably the crusades Italy (Puglia). It could be important in the geography of played an important role in the spread of leprosy in Italy. the spread of leprosy. In recent years, the description and In particular, the route of the First Crusade (1096–1099) 6 Journal of Anthropology interested southeastern Italy (see Figure 6), and it opened [11] S. Mazzi, Salute e Societa Durante il Medioevo,LaNuova Editrice, Milan, Italy, 7th edition, 2010. an important trade and military route toward the Near East [12] C. Rawcliffe, Leprosy in Medieval England, Boydell Press, [48]. According to this hypothesis, leprosy present in North Woodbridge, UK, 2006. and Central Italy in the first millennium AD spread in [13] A. K. Wilbur, J. E. Buikstra, and C. Stajanowski, “Mycobac- the first centuries of the second millennium to the South, terial disease in North America: an epidemiological test of favoured by this great flow of soldiers, traders, clerics, and Chaussinand’s cross-immunity hypothesis,” in The Past and adventurers. On the other hand, the scarce evidences of Present of Leprosy. 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The First Probable Case of Leprosy in Southeast Italy (13th-14th Centuries AD, Montecorvino, Puglia)

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Copyright © 2012 Mauro Rubini et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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Hindawi Publishing Corporation Journal of Anthropology Volume 2012, Article ID 262790, 7 pages doi:10.1155/2012/262790 Research Article The First Probable Case of Leprosy in Southeast Italy (13th-14th Centuries AD, Montecorvino, Puglia) 1, 2 2 2 2 1 Mauro Rubini, Valentina Dell’Anno, Roberta Giuliani, Pasquale Favia, and Paola Zaio Anthropological Service, SBAL, Via Pompeo Magno 2, 00193 Rome, Italy Department of Archaeology, Foggia University, Piazza Civitella 2, 71121 Foggia, Italy Correspondence should be addressed to Mauro Rubini, antropologiasal@libero.it Received 21 March 2012; Revised 3 June 2012; Accepted 25 June 2012 Academic Editor: Scott M. Fitzpatrick Copyright © 2012 Mauro Rubini et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. In 2008, during an archaeological excavation on the medieval site of Montecorvino (Foggia, Puglia, Italy), ten individuals were found buried near the principal church. The tombs were dated to the 13th-14th centuries AD, except for one attributable to the 11th century AD. The individual from tomb MCV2 shows some bone changes in the rhinomaxillary area. The most probable diagnosis is that she suffered from a type of near-multibacillary leprosy. Although leprosy has been documented in Italy from the first millennium BC and well described in the first millennium AD, its presence seems to be confined to Northern and Central Italy. This is the first case of leprosy in southeastern Italy and the second in Southern Italy overall. At the moment, the interesting datum is that leprosy seems to appear in Southern Italy only after the first millennium AD. All this could be because of the First Crusade with the opening of new trade and pilgrimage routes to the Near East or simply because other cases of leprosy have still not been found in osteoarchaeological context. 1. Introduction over 60 [6, 7]. Response to the disease is highly variable, and the immune status of each infected person determines Leprosy, or Hansen’s disease, is a chronic infection caused the type and severity of pathological change [8]. In its most by an unculturable pathogen, Mycobacterium leprae.It severe form, multibacillary (MeSh-lepromatous) leprosy, affects the peripheral nervous system primarily, especially both the skin lesions and the peripheral nerve damage the extremities (neural leprosy), and secondarily involves become generalised and symmetrical in nature. Those with the skin, nasal tissues, and bones [1]. As is well known, high immune status are more likely to develop paucibacillary Mycobacterium leprae is a highly infective bacterium with low (MeSh-tuberculoid) leprosy, where only a small number pathogenicity; that is, its ability to induce clinical disease is of skin lesions develop and peripheral nerve damage is low [2]. The bacteria affect the motor, sensory, and auto- asymmetrical. Having reached endemic status in mediaeval nomic nervous systems. M. leprae accumulates principally Europe, leprosy declined in the postmediaeval period about in the extremities of the body, where it resides within the the 14th century AD [9–11]. The decline of leprosy in macrophages and infects the Schwann cells of the peripheral Western Europe implied a complex web of factors, especially nervous system. Lack of myelin production by infected medical, environmental, social, and legal [12]. Under the Schwann cells and their destruction by host-mediated biomedical aspect, this decline probably may have been immune reactions lead to nerve damage, sensory loss, and caused by the cross-infection between leprosy and tuberculo- the disfiguration that, sadly, are the hallmarks of leprosy [3]. sis [13–15], although this possibility is debate still today and, Primary transmission occurs through droplet infection [4]. the exact relationship between TB and leprosy still remains The incubation period is very long (in some cases 20 years) unclear [16]. before clinical signs and symptoms become apparent [5]. In Italy, the skeletal documentation on leprosy is scarce. Leprosy can affect all age groups and seem to prefer male sex, According to the osteoarchaeological evidence, it was present although it is often first observed before the age of 20 and in the Northeast in the first millennium BC [17], in Central 2 Journal of Anthropology Figure 1: Geographic location of the site. Italy during the Roman period near Rome with the cases of Palombara [18] and Martellona [19] and in the early Middle Ages with the cases of Campochiaro Vicenne [20]and Campochiaro Morrione [21]. Furthermore, there is another late medieval case in South Italy [22]. Leprosy is rarely found today in Europe but is still a significant disease worldwide, principally in Southeast Asia, Africa, and South America (Brazil) [23]. The aim of this study is to investigate and discuss the skeleton of an individual from the medieval church of Montecorvino that shows interesting rhinomaxillary changes. Figure 2: Tomb MCV2. 2. Material and Methods In 2008, during an archaeological excavation in the medi- on the skeleton was effected according to the suggestions aeval town of Montecorvino (Foggia, Puglia—Figure 1), ten of Andersen and Manchester [32], Roberts and Manchester skeletons were found buried in graves near the principal [33], and Nunzi, and Massone [5]. church. The chronology obtained from the gravegoods is 13th-14th century AD, except for one dated to the 11th 3. Description of the Skeleton century AD. The skeleton under study was found in a tomb that was built with bricks near the perimetral wall of the MCV2 is a skeleton attributable to a female aged 35–40 years. church (Figure 2) and was found without covering. The The examination of the skeletal remains shows some bone code number of this individual is MCV2. The only object changes in the rhinomaxillary region. The alveolar process of found with it is an iron belt-buckle, which was dated by its the maxilla shows an intense erosive activity in the anterior particular shape to the end of the 13th century AD (Favia, side with loss of bony tissue producing the exposure of the personal communication). The skeletal remains are quite cavity of the root of the left and right central incisors and well preserved but unfortunately incomplete (Figure 3). The right lateral incisors, which are all absent (see Figure 4(a)). diagnosis of sex and age at death was carried out according The loss of teeth was surely antemortem because the alveolar to the standard suggested by Buikstra and Ubelaker [24]. In cavities show a discrete atrophy. In the left side, a damage particular, sex determined by the morphology of the os coxae postmortem is present in the alveolar portion of the lateral [25–27]and cranium[28]. Age at death was calculated on the incisor and canine. Although the area is damaged in the morphological changes of the pubic symphyseal face [29], on left side, the anterior nasal spine could be resorbed (see the auricular surface of the os coxae [30] and on the cranial Figure 5(a)) because of visible traces of little bone neoforma- suture closure [31]. The screening of the traces of leprosy tion. There is a symmetrical resorption and remodelling of Journal of Anthropology 3 (a) Figure 3: Bones present (grey) in individual MCV2. the normally sharply defined inferior margins of the piriform aperture with irregular rounding of the external profile (see Figure 5(a)), while the internal nasal surface is pitted (Figure 4(b)). The right lateral margin is smooth and thick in (b) appearance, while the left is damaged. Furthermore, the left and right inferior nasal conchae were partially resorbed. In Figure 4: (a) Alveolar process of the maxilla with an intense erosive conjunction with the remodelling of the margins of the nasal process in anterior part and loss of bony tissue with exposure of the aperture, the alterations of the intranasal structures (that are tooth roots in the right side and of the left central incisor (black not damaged) seem in the appearance of a wide, semiempty arrows). Furthermore, the margins of the piriform aperture are cavity (see Figure 5(a)). The alveoli of the anterior teeth rounded (white arrows). (b) Particular of the nasal cavity. On the shows an irregular shape in the lingual edge, and in the hard bony wall there is presence of pitting (arrows). palate there is an intense presence of pitting that involves also the alveolar cavity of the right and left molars (Figure 5(b)). In the postcranial skeleton only a light periostitis is present infections caused by spirochetes of the genus Treponema. on the tibiae in mesiodistal position. Excluding pinta, which does not affect bones,in the tertiary stage these diseases can involves the skeleton, and they tend 4. Differential Diagnosis to be associated with inflammatory bony changes accompa- There are various pathological conditions that could lead nied by extensive bone regeneration, often resulting in alter- to the destruction of bone in the rhinomaxillary region ation of the bone morphology and in some cases destruction [34]. These disorders include granulomatous diseases such of the nasal-palatal area may occur [34]. Yaws is typical of the as sarcoidosis and treponemal diseases and fungal infections humid tropical regions of South America, Africa, Asia, and such as aspergillosis and mucormycosis (phycomycossis), acti- Oceania. In this disorder, the last stage can be characterized nomycosis (a bacterial rather than a true fungal disease), and by widespread bone, joint, and soft tissue destruction, which lupus vulgaris (tuberculosis of the facial skin and soft tissue). may include extensive destruction of the bone and cartilage Sarcoidosis is a granulomatous disease of unknown aeti- of the nose (rhinopharyngitis mutilans or “gangosa”). Joints ology. Like leprosy, it tends to affect the phalanges of the may stiffen, and chronic osteitis and periostitis can lead to fingers and toes, causing lytic lesions and no reactive bone deformed leg bones (sabre tibiae). Bejel (endemic syphilis) formation [34]. However, in the skull, it causes mainly is present in nomadic populations of the Sahel and more in the destruction of the nasal bones while only rarely of the general in the desert region of Africa, in the Middle East, anterior nasal spine and never of the crest [35]. Thus, we can Central Australia, and Asia. Periostitis of the leg bones is exclude this disease as an explanation for the bony lesions in commonly seen. The involvement of the skull is very rare, our specimen. In view of the reconsideration of the origin with gummas of the soft palate and nose developing in the of treponemal disease in the Old World, we may also discuss last stage [35]. The “gangosa” condition may occur rarely. this group of pathologies since, like leprosy, they can lead to In venereal syphilis, the most commonly affected bones are the destruction of the nose and to bony changes in the lower (in order of importance) tibia, frontal and parietal (with limbs [34]. Treponemal diseases include syphilis (venereal caries sicca on the outer tables), nasal-palatal region, clavicle, and endemic), yaws and pinta, which are granulomatous sternum, vertebrae, fibula, femur, humerus and radius, and 4 Journal of Anthropology Mucormycosis (phycomicosysis) is a rare invasive fungal infection, which tends to affect people who have poorly controlled diabetes. It results in black, dead tissue in the nasal cavity and blocks in the blood supply to the brain, leading to neurological symptoms, such as headaches and blindness [38]. Mucormycosis attacks the nasal cavity with involvement of the paranasal sinuses and their walls [34]. A diagnosis of aspergillosis and mucormycosis can be ruled out, because in no case is the unilateral and bilateral perforation of the hard palate present. Furthermore, no traces of this pathology are present in the orbit region of the Montecorvino skeleton. Actinomycosis produces effects on the cervicofacial area; bone involvement is rare. When affected, the mandible rather than the maxilla is more involved [34, 39]. This is not the case with our individual. (a) Lupus vulgaris is a chronic tubercular infection of the skin involving soft yellow swellings, ulcers, and abscesses. Long-standing tuberculosis of the facial skin and soft tissues can lead to the destruction of the nasal bones [35]. The anterior alveolar process, however, is rarely affected [37], which discounts lupus vulgaris as a diagnosis. Given the localization of the lesions in our sample, the most likely diagnosis is multibacillary leprosy [32, 33, 37, 40]. Some bone changes are present in the rhinomaxillary region. The enlargement and rounding of the piriform aperture, the cicatrisation of the lower margin of the inferior nasal aperture, and erosion of the alveolar margin accompanied by the loss of the front teeth could be changes seen in leprosy referred to as rhinomaxillary syndrome [32, page 122]. Although the area is damaged, also the resorption of the ante- rior nasal spine could be present. Furthermore, the presence of pitting shows a presence of a chronic inflammation in the (b) nasal-palatal region. Deformity of the nasal-maxillary facial Figure 5: (a) Particular of the possible resorption of the anterior structures results from primary skeletal infection by Myco- nasal spine with bone neoformation (black arrows) in the right part bacterium leprae [34, 41], and alterations of the hands and (the left is partially damaged) and remodelling of the margins of the feet (especially the fifth metatarsal bone, where the same nasal aperture with presence of bony neoformation (white arrows). nerves that enervate the fibula are present) are secondary (b) Hard palate. On the surface an intense pitting process is present to pathological changes in the peripheral nerves with conse- that involve also the alveolar cavity of the left and right molars. quent loss of sensory and motor functions [42]. These last osteological markers are not present in our sample, but a light periostitis in the lower limbs is present also if aspecific. ulna. The teeth may also be involved, showing a screw- Periostitis, also known as periostalgia, is a clinical condition driver shape (Hutchinson teeth). No specific involvement of caused by inflammation of the periosteum, a layer of con- the feet is observed in this pathology [34, 35]. The most nective tissue that surrounds bone. The condition is generally common bones afflicted during the tertiary stage of the chronic and is marked by tenderness and swelling of the bone and an aching pain [35]. It could be due to scurvy, fractures, disease are the tibia (with sabre-shaped deformity) and the skull. According to Ortner [34], the calvarial lesions are the and infectious diseases such as, for example, treponematosis most specific diagnostic features. Although the nasal cavity and leprosy [35]. In this case, in absence of fractures or scurvy it is probably secondary and associated with an is often enlarged, producing the characteristic “saddle nose,” the nasal spine is usually spared [36]. Furthermore, the infectious disease. anterior alveolar change process is uncommon in yaws and syphilis [37, page 297]. Therefore, the pathogenetic picture 5. Discussion and Conclusions of our skeleton does not correspond to that of treponemal diseases. The study of the pathological changes of the skeleton from Aspergillosis is marked by inflammatory granulomatous the church at Montecorvino shows that this individual lesions in the skin, ear, orbit, nasal sinuses, lungs, and probably suffered from a type of near multibacillary leprosy. sometimes bones and meninges. It affects the paranasal When leprosy affects the skeleton, a number of specific and sinuses and orbit or the anterior cranial fossa [35]. nonspecific bony and osteoporotic changes occur during Journal of Anthropology 5 The route of the First Crusade (1096–1099) Kilometers Figure 6: The route of the First Crusade (1096–1099). pathogenesis [43]. Specific bony changes are caused by inva- publication of leprosy cases in Italy has increased (for a sion of the tissues by M. leprae, which is why facial changes survey see [21]). During the first millennium AD, there are are seen; secondary bone changes are a result of peripheral no osteological cases found in southern Italy, although many nerve involvement (hands, feet and lower leg bones) and lead prehistoric and historic skeletal collections have been studied to sensory nerve anaesthesia, trauma to the hands and feet by anthropologists and palaeopathologists. At present, the because people cannot feel the trauma, then ulceration of the most ancient cases known are that of Henry VII in Calabria feet and hands, then secondary infection of the bones of the (southwest Italy) dated to the thirteenth century AD [22] hands and feet [5]. According to Nunzi and Massone [5], the and now that of Montecorvino (southeast Italy) dated major skeletal foci of leprosy are the face and small tubular to the thirteenth-fourteenth century AD. The spread of bones of the hands and feet. Rarely also other bones may be leprosy in Southern Italy could be subsequent to the first affected [34]. The mediaeval individual from Montecorvino wave that afflicted Northern and Central Italy during the shows some traces of leprosy, with a possible rhinomaxillary first millennium AD [21]. In fact, during this period, the syndrome. Although pathogenesis of the skeletal changes in phenomenon of the “Migration of the Peoples” or barbarian leprosy is generally difficult to assess in osteoarchaeological invasions produced an increase in epidemic diseases in Italy remains, Ortner [34] observed that the presence of destruc- [18]. An explanation for the presence of leprosy only in tive and proliferative lesions could indicate the infectious Northern and Central Italy in the first millennium AD could phase (chronic or acute) of the disease, without specifying be found in the pattern of Monot et al. [46] based on the use the pathological process that caused the bone changes. In of comparative genomics. Monot et al. demonstrated that the fact, Mycobacterium leprae prefers cool environments, and single clone responsible for leprosy can be traced by analysis the distribution of the lesions in multibacillary leprosy is of rare single-nucleotide polymorphisms (SNPs). They have associated with the cooler, exposed areas of the skin and obtained four permutations (types) of SNPs distributed mucous membranes like that within or under the nose or throughout the world. According to these results, leprosy more generally of the face, which constitutes the elective seem to have originated in eastern Africa or the Near East site of aggression of the disease [1]. Leprosy does not cause and spread with successive human migrations. According to death; rather, people usually die of TB [13, 14]. Therefore, the the scheme of Monot et al., from about 40,000 years BP different manifestations of the disease in osteoarchaeological the disease’s path was the same as that of the route of the remains are not homogeneous but can show numerous human populations obtained from genetic and anthropo- variants [34]. Furthermore, the last consideration is that logical data and was directed from Central Asia/Eastern MCV2 was buried in the normal cemeterial area of the Europe to Western Europe [47]. This human flow would church. This is not infrequent during this period. Usually seem to be uninterrupted during the first millennium AD individuals with leprosy were buried in normal cemetery preferring terrestrial way [21]. This last observation could [9, 20, 21, 44, 45]. be confirmed by the geographical location of the Italian The mediaeval individual from Montecorvino is the first osteoarchaeological evidences. case of leprosy described in the literature in southeastern After the first millennium AD, probably the crusades Italy (Puglia). It could be important in the geography of played an important role in the spread of leprosy in Italy. the spread of leprosy. 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Journal of AnthropologyHindawi Publishing Corporation

Published: Aug 5, 2012

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