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Cancrum oris in a boy with Down syndrome

2011; Elsevier BV; Volume: 64; Issue: 6 Linguagem: Inglês

10.1016/j.jaad.2009.08.048

1097-6787

Serena Lembo, Chiara De Leonibus, Maria Grazia Francia, Claudio Lembo, Fabio Ayala,

Oral and gingival health research

To the Editor: A 17-year-old Italian male with Down syndrome presented to the dermatology department with a 2-cm ulcerative lesion on the right tip of his tongue (Fig 1). The onset was about 7 days earlier with mild swelling. The patient's parents described a persistent vesicular eruption in the same area during the previous 2 months. They attributed it to biting and self-inflicted injuries and treated it with multiple but inefficacious drugs (nystatin, metisoprinol, diflucortolone, and chloramphenicol). The physical examination revealed that the right submandibular lymph nodes were slightly enlarged and tender. Fever and malaise were not reported, and neither were any other symptoms. Routine hematologic parameters were normal, apart from a chronic low leukocyte count. These findings were similar to his previous laboratory examinations. His medical history, as reported by the parents, was negative for any kind of recurrent infection. He was otherwise in good health and had never been institutionalized. An HIV test was negative. An oral swab revealed significant growth of multiple anaerobic bacteria. Cancrum oris was strongly suspected, and a 3-mm punch biopsy of the lesion was performed. Histologic examination revealed a nonspecific acute process characterized by a heavy inflammatory infiltrate mainly comprised of neutrophilic abscesses and blood vessel endothelial swelling, suggestive of bacterial infection. As indicated by microorganism culture and sensitivity tests, an intramuscular dose of lyncomicin 600 mg (2 mL) twice per day was initiated, along with oral doses of multivitamin supplements. Dequalinium chloride was applied topically three times per day. After 10 days, the oral lesion was dramatically improved and the ulceration had almost completely resolved (Fig 2).Fig 2After 10 days of antibiotic therapy, the patient experienced dramatic improvement.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Cancrum oris, also known as noma, is an acute gangrenous stomatitis that may affect the oral mucosa, the labiomental, and/or the labiomaxillary structures. The term “νoμα” (noma) is derived from the Greek νoμη, which means “to graze or to devour,” indicating massive necrosis and possible severe sequelae, such as facial disfigurement, trismus, oral incontinence, and speech problems.1Marck K.W. Cancrum oris and noma: some etymological and historical remarks.Br J Plast Surg. 2003; 56: 524-527Abstract Full Text Full Text PDF PubMed Scopus (16) Google Scholar The global yearly incidence of cancrum oris is 140,000 cases, as estimated by the World Health Organization; it occurs mainly in less developed regions, such as sub-Saharan Africa.2Bourgeois D.M. Leclercq M.H. The World Health Organization initiative on noma.Oral Dis. 1999; 5: 172-174Crossref PubMed Scopus (48) Google Scholar Noma is seen predominantly in children between 1 and 4 years of age, although late stages can occur in adolescents and adults. Infants are less susceptible because of the protection conferred by breastfeeding.3Enwonwu C.O. Falkler Jr., W.A. Phillips R.S. Noma (cancrum oris).Lancet. 2006; 368: 147-156Abstract Full Text Full Text PDF PubMed Scopus (137) Google Scholar Most recent cases of cancrum oris reported in developed countries have been related to poor oral hygiene, malnutrition, dehydration, blood dyscrasias, or other debilitating conditions, such as intense immunosuppressive therapy, AIDS, or other viral infections.4Enweon W.U. Epidemiological and biochemical studies of necrotizing ulcerative gingivitis and noma (cancrum oris) in Nigerian children.Arch Oral Biol. 1972; 17: 1357-1371Abstract Full Text PDF PubMed Scopus (95) Google Scholar Polymicrobial growth is involved in noma and the bacterial enzymes are responsible for host tissues necrosis. Cellular immunodepression in patients affected with Down syndrome is considered responsible for bacterial infections which, in turn, play a major role in tissue necrosis.Early antibiotic therapy is therefore suggested, including oral ampicillin/gentamicin or chloramphenicol, plus metronidazole, in association with topical chlorhexidine digluconate solution for mouth rinsing. The mortality rate has declined from 80-90% to 8-10% as a result of antibiotic use.5Tempest M.N. Cancrum oris.Br J Surg. 1966; 53: 949-969Crossref PubMed Scopus (133) Google Scholar Exclusive breastfeeding for at least 6 months should be promoted in poor communities to increase immunoprotection.4Enweon W.U. Epidemiological and biochemical studies of necrotizing ulcerative gingivitis and noma (cancrum oris) in Nigerian children.Arch Oral Biol. 1972; 17: 1357-1371Abstract Full Text PDF PubMed Scopus (95) Google Scholar Recurrence is extremely rare. Even with appropriate antibiotics, aggressive debridement or flaps may be necessary to close the defect or large orocutaneous fistulae provoked by extensive tissue necrosis.In our patient, the incredibly quick resolution of such a destructive clinical picture highlights the importance of rapid diagnosis and the initiation of specific therapy. In fact, early treatment with specific antibiotics can reverse the negative prognosis of the disease. To the Editor: A 17-year-old Italian male with Down syndrome presented to the dermatology department with a 2-cm ulcerative lesion on the right tip of his tongue (Fig 1). The onset was about 7 days earlier with mild swelling. The patient's parents described a persistent vesicular eruption in the same area during the previous 2 months. They attributed it to biting and self-inflicted injuries and treated it with multiple but inefficacious drugs (nystatin, metisoprinol, diflucortolone, and chloramphenicol). The physical examination revealed that the right submandibular lymph nodes were slightly enlarged and tender. Fever and malaise were not reported, and neither were any other symptoms. Routine hematologic parameters were normal, apart from a chronic low leukocyte count. These findings were similar to his previous laboratory examinations. His medical history, as reported by the parents, was negative for any kind of recurrent infection. He was otherwise in good health and had never been institutionalized. An HIV test was negative. An oral swab revealed significant growth of multiple anaerobic bacteria. Cancrum oris was strongly suspected, and a 3-mm punch biopsy of the lesion was performed. Histologic examination revealed a nonspecific acute process characterized by a heavy inflammatory infiltrate mainly comprised of neutrophilic abscesses and blood vessel endothelial swelling, suggestive of bacterial infection. As indicated by microorganism culture and sensitivity tests, an intramuscular dose of lyncomicin 600 mg (2 mL) twice per day was initiated, along with oral doses of multivitamin supplements. Dequalinium chloride was applied topically three times per day. After 10 days, the oral lesion was dramatically improved and the ulceration had almost completely resolved (Fig 2). Cancrum oris, also known as noma, is an acute gangrenous stomatitis that may affect the oral mucosa, the labiomental, and/or the labiomaxillary structures. The term “νoμα” (noma) is derived from the Greek νoμη, which means “to graze or to devour,” indicating massive necrosis and possible severe sequelae, such as facial disfigurement, trismus, oral incontinence, and speech problems.1Marck K.W. Cancrum oris and noma: some etymological and historical remarks.Br J Plast Surg. 2003; 56: 524-527Abstract Full Text Full Text PDF PubMed Scopus (16) Google Scholar The global yearly incidence of cancrum oris is 140,000 cases, as estimated by the World Health Organization; it occurs mainly in less developed regions, such as sub-Saharan Africa.2Bourgeois D.M. Leclercq M.H. The World Health Organization initiative on noma.Oral Dis. 1999; 5: 172-174Crossref PubMed Scopus (48) Google Scholar Noma is seen predominantly in children between 1 and 4 years of age, although late stages can occur in adolescents and adults. Infants are less susceptible because of the protection conferred by breastfeeding.3Enwonwu C.O. Falkler Jr., W.A. Phillips R.S. Noma (cancrum oris).Lancet. 2006; 368: 147-156Abstract Full Text Full Text PDF PubMed Scopus (137) Google Scholar Most recent cases of cancrum oris reported in developed countries have been related to poor oral hygiene, malnutrition, dehydration, blood dyscrasias, or other debilitating conditions, such as intense immunosuppressive therapy, AIDS, or other viral infections.4Enweon W.U. Epidemiological and biochemical studies of necrotizing ulcerative gingivitis and noma (cancrum oris) in Nigerian children.Arch Oral Biol. 1972; 17: 1357-1371Abstract Full Text PDF PubMed Scopus (95) Google Scholar Polymicrobial growth is involved in noma and the bacterial enzymes are responsible for host tissues necrosis. Cellular immunodepression in patients affected with Down syndrome is considered responsible for bacterial infections which, in turn, play a major role in tissue necrosis. Early antibiotic therapy is therefore suggested, including oral ampicillin/gentamicin or chloramphenicol, plus metronidazole, in association with topical chlorhexidine digluconate solution for mouth rinsing. The mortality rate has declined from 80-90% to 8-10% as a result of antibiotic use.5Tempest M.N. Cancrum oris.Br J Surg. 1966; 53: 949-969Crossref PubMed Scopus (133) Google Scholar Exclusive breastfeeding for at least 6 months should be promoted in poor communities to increase immunoprotection.4Enweon W.U. Epidemiological and biochemical studies of necrotizing ulcerative gingivitis and noma (cancrum oris) in Nigerian children.Arch Oral Biol. 1972; 17: 1357-1371Abstract Full Text PDF PubMed Scopus (95) Google Scholar Recurrence is extremely rare. Even with appropriate antibiotics, aggressive debridement or flaps may be necessary to close the defect or large orocutaneous fistulae provoked by extensive tissue necrosis. In our patient, the incredibly quick resolution of such a destructive clinical picture highlights the importance of rapid diagnosis and the initiation of specific therapy. In fact, early treatment with specific antibiotics can reverse the negative prognosis of the disease.