Infectious disease of unknown etiology: Noma (Cancrum Oris)

Noma is an ulcer-necrotic gangrenous infection. Its name comes from the Greek verb (to devour). Noma common affects children between ages three and twelve years old. The physical signs of Noma are gingival and cheek edema produced by the destruction of the mouth’s epithelium, connective tissue and the papillae. Necrotizing stomatitis, leads to the loss of periodontal attachment and bone tissue damage at the alveolar margin in the premolar to molar and mandibular incisor regions extending to the labiogingival fold and on to the mucosal surface of the cheek and lip¹.

The loss of the oral facial tissue is very diverse and can vary in size; the most affected areas are the nose, upper lip, pre maxilla and the infraorbital margin¹. The sequela of acute noma can include displacement of teeth, intense scarring, bony fusion between mandible and maxilla, trismus, defective speech and nasal regurgitation if the maxilla is lost ². Survivors of the acute phase have to deal with disfigurement and functional impairment and psychological traumas as well as speech impediments².

Acute necrotizing gingivitis (AGN) is considered one of the main precursors of Noma, as well as the herpes virus and measles. The influence of Herpes virus in Noma is through the association between the cytomegalovirus and periodontal diseases¹. During the infection of cytomegalovirus the ratio of CD 4 helper cells is bigger than the CD8 cytotoxic cells, which leads to a weak immune response and the presence of secondary infections. In addition, cytomegalovirus infection may cause the increase of IL-1, which is a potent bone resorptive cytokines^2. The correlation between cytomegalovirus and ANG is clear because the herpetic oral lesion damages the barrier of the oral mucosa and reduces the immune system response which leads in to bacterial proliferation that may lead to ANG in malnourish children.

This disease is preventable if diagnosed and treated in the early stages; however the countries where the incidence of the disease is high do not have access to clean water and oral health prevention programs to reduce the prevalence of the disease, and children with an advanced case of Noma died within three to four months.

References:

1. WA Falkler Jr, CO Enwonwu, EO Idigbe. Microbiological understandings and mysteries of noma (cancrum oris) Article first published online: 28 JUN 2008 DOI: 10.1111/j.1601-0825.1999.tb00081.

2. Enwonwu CO , Falkler WA , Idigbe EO . Oro-facial gangrene (noma/cancrum oris): pathogenetic mechanisms. Crit Rev Oral Biol Med 2000;11:159-171 CrossRef | Medline.