First AD

Second AD

An undernourished child presents with a facial lesion present for some months (

). The problem started as a gingival ulcer with facial edema and progressed to a necrotic black escar. After sloughing of the escar and the external cheek skin, the area finally healed with a scar and retraction over the course of the next weeks.

A. ANUG (acute necrotizing gingivostomatitis 

Noma (cancrum oris) is a devastating orofacial gangrene promoted by extreme poverty, lending it the name “the face of poverty.”

 It is seen worldwide, but is most prevalent in sub-Saharan Africa.

 Acute noma is primarily seen in children ages 1-4, with 90% of cases developing before the age of 10.

The disease starts as a gum lesion, evolving to facial edema and rapidly eroding through soft tissue and bone.

 If not treated adequately, noma has a mortality rate as high as 90% within 1-2 weeks due to sepsis. Patients who do survive this destructive disease are left with severe facial disfigurement, oral incontinence, trismus, and speech problems.

The World Health Organization designated noma a health priority in 1994, estimating a worldwide incidence of 140,000 cases yearly and prevalence of 770,000 people living with devastating sequelae. These numbers are thought to be underreported because of the lack of official monitoring, high mortality leaving many cases undiscovered, and sociocultural obstacles where afflicted children are shamefully hidden.

 The disease has almost disappeared in developed countries, primarily because of improved standards of living and modern antibiotics, with mortality reduced to 8-10%.

Unfortunately, in some communities, noma is believed to be a curse on the family, so medical attention is not sought until much later in the disease process.

 Once the gangrenous process has perforated the cheek, antibiotics can only lower the risk of death. The fibrous scarring and disfiguring effects will remain.

is multifactorial-a complex interaction between infection, malnutrition, and a compromised immune system.

 Initial stages and prodromes of the disease are not well known since medical care is not sought during acute phases.

Most experts agree that acute necrotizing gingivostomatitis (ANUG) and gingival edema rapidly progressing to necrosis, in children in less developed countries can lead to noma. Poor oral hygiene has been identified as a factor.

Almost all patients with noma have a recent history of debilitating infection, including chickenpox, small pox, typhoid fever, typhus, diphtheria, leishmaniasis, pneumonia, AIDS, and with malaria and measles being the most common.

 Scientific evidence in in-vitro studies has shown measles results in a temporary immunosuppressed state similar to AIDS.

Protein-energy malnutrition or vitamin deficiencies affect the immune system as well as tissues with rapid turnover, such as gingival mucosa. The patient is left with compromised immunity and gingival tissue susceptible to oral pathogens.

 While many bacteria have been implicated, 

 have been noted as key pathogens in noma.

 Further research is needed to identify the pathophysiology and exact causative agents in the disease.

include a recent history of debilitating disease or exanthematous fever, oral ulcer with bone exposure, fetid breath, excess salivation and stunted growth or wasting.

  Patients with acute noma may also have fever, anorexia, tachycardia and tachypnea. A recent medical history of recurrent diarrhea, and infection with viruses and parasites is also seen. Severe anemia, low white-blood-cell count and hypoalbuminemia are common, with growth retardation as the most prominent feature. Variants of noma include noma neonatorum,

 a pseudomonal that occurs primarily in preterm infants and noma pudendi.

 The differential diagnosis includes malignant oral lesions (midline lethal granuloma), leishmania, dental sinus, buruli ulcer, rhinomaxilary syndrome, granulomatosis with polyangiitis and gangosa, most rare in children.

of the disease, management is geared toward improving the patient’s overall health status. Antibiotics, rehydration, nutritional rehabilitation and vitamins are given to hasten these efforts. Broad-spectrum antibiotics with coverage for aerobic and anaerobic oral flora are prescribed when a specific pathogen has not been identified.

 Some researchers believe metronidazole to be adequate treatment since noma is predominantly associated with anaerobic species.

Invasive intraoral interventions, besides the removal of loose teeth, are contraindicated because of the risk of exacerbating the lesion.

 during the healing process to prevent strictures due to fibrous scarring.

 Reconstructive surgery is considered after the lesion is well demarcated and healing, usually not earlier than 1 year after the onset of disease. Types of surgical repair vary on a case-by-case basis depending on the extent of the defect.

Noma is primarily encountered in remote, underprivileged and illiterate communities where parents and many health personnel do not know much about the disease. Because of this, community education is a key player in prevention and early detection of the disease.

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 Enwonwu CO, Falkler WA, Jr, Phillips RS. Noma (cancrum oris). Lancet. 2006;368:147-56.

 Adolph, HP, Yugueros P, Woods JE. Noma: a review. Ann Plast Surg, 1996;37:657-68.

. Bourgeois DM, Leclercq MH. The World Health Organization initiative on noma. Oral Dis. 1999;5:172-4.

 Tempest MN. Cancrum oris. Br J Surg, 1966;53:949-69.

 Berthold P. Noma: a forgotten disease. Dent Clin North Am. 2003;47:559-74.

 Baratti-Mayer D, Pittet B, Montandon D, et al. Noma: an "infectious" disease of unknown aetiology. Lancet Infect Dis;20033:419-31.

 Salmi AA. Suppression of T-cell immunity after measles infection: is the puzzle solved? Trends Microbiol. 1997;5:85-6.

Skin Diseases

What disease, known in antiquity but largely eradicated from the developed world, caused this severe facial scarring? 

A Young Child with a Devastating Facial Lesion