-
catarrhal stomatitis : transitory inflammation of the oral mucosa,
sometimes with gingivitis, erythema, swelling, and occasionally epithelial
desquamation; believed to be caused by the oral bacterial flora.
-
gonorrheal or gonococcal stomatitis : gonorrhea
of the oral cavity, usually transmitted by orogenital contact, characterized
by a linear or flattened eruption associated with redness, itching, and
burning of the mucosa
-
stomatitis exanthematica : stomatitis secondary to an exanthematous
disease.
-
stomatitis scarlatina : a condition of the oral mucosa seen in scarlatina
(scarlet fever),
characterized in the early stages by fiery red coloration, congestion,
and exudate of the throat, and by strawberry tongue and raspberry tongue
in the later stages.
-
Koplik spots : measles
virus
-
syphilitic stomatitis : stomatitis due to systemic syphilis
-
noma / cancrum
oris / gangrenous stomatitis : a severe gangrenous process usually
seen in debilitated and severely malnourished children in developing nations,
beginning as a small vesicle or ulcer on the gingiva that rapidly becomes
necrotic and spreads to destroy large areas of the buccal and labial mucosa
and tissues of the face, resulting in severe disfigurement or even death.
"Noma" derives from the Greek word nemo, meaning "to graze" or "to devour."
The disease dates back to antiquity, but its name was first used by a Danish
physician in 1680 and was meant to underscore its astonishingly rapid developmentref.
To Hausa communities in Nigeria, it is known as ciwon iska an
unexplained, sudden illness linked to the spirits
Epidemiology : the incidence
of acute noma peaks at 1-4 years of age, although late stages occur in
adolescents and adults
ref.
Noma was common in Europe and North America until the early 20th century,
when it essentially disappeared from developed countries, except for cases
found in the concentration camps of Bergen-Belsen and Auschwitz and, more
recently, in association with intensive immunosuppressive therapy, in some
patients with
HIV-1
infection, and in Native American children with severe combined immunodeficiency
syndrome. Yet the disease is still prevalent in developing countries. The
World Health Organization (WHO) has compiled a global picture of reported
cases :
Although African countries are the most affected, Asia
and Latin America are not exempt. Before 1980, many sub-Saharan countries
had underdeveloped health-reporting systems. Since the 1990s, awareness
of noma has increased, and many countries have developed control plans.
Sporadic cases have also been reported in higher-income countries, including
the USA and some Western European countries, where noma-like lesions may
be associated with HIV infection or AIDS. Accurate epidemiologic data are
lacking because noma occurs predominantly in poor communities that do not
keep health records and, frequently, have a nomadic lifestyle. Also, the
disease is often hidden by families who consider it an evil omen. In 1998,
the WHO estimated the annual global incidence at 140,000 and the associated
mortality at 70 to 80% among persons who are not treated promptly. A more
recent report estimates an annual incidence of 25,000 in the developing
countries bordering the Sahara
ref.
But these estimates reflect the tip of the iceberg, since it is believed
that < 10% of affected persons seek medical care.
Aetiology : noma thrives
in communities characterized by extreme poverty, severe malnutrition (particularly
micronutrient deficiencies), unsafe drinking water, poor sanitation, poor
oral health practices, high infant mortality, limited access to high-quality
health care, and a high prevalence of low birth weight, attributable primarily
to intrauterine growth retardation. In these communities, families may
share their overcrowded, poorly ventilated living quarters and sources
of drinking water with domestic animals, and foods for infants may be prepared
under unhygienic conditions. All these factors promote chronic and recurrent
infection-induced immunostimulation by environmental antigens. Noma is
usually preceded by a debilitating illness, such as
measles,
malaria,
diarrhea,
tuberculosis,
or
necrotizing ulcerative
gingivitis. Noma is a polymicrobial opportunistic infection, but there
is no consensus regarding the causative microorganisms. Studies point to
..., which may enter children's mouths through water
and food contaminated with animal feces
ref.
Pathogenesis of noma involves
a complex interaction among malnutrition, immune dysfunction, and infection
with endemic viruses that creates a "staging period" characterized by impaired
oral mucosal immunity, defective structural integrity of the oral mucosa,
selective overgrowth of pathogenic microorganisms, increased oxidative
stress, and a shift from an inflammatory to an antiinflammatory cytokine
profile, among other changes. An intraoral ulcer develops and probably
provides a site of entry for a triggering microorganism. In a final invasive
phase, the lesion spreads rapidly, probably owing to the presence of necrotizing
toxins or tissue-destroying enzymes and inflammatory mediators. Still puzzling
is the observation that in the same households, oral mucosal ulcers progress
to noma in only a small subgroup of severely malnourished children. At
least 85% of Nigerian children under 3 years of age who have noma also
have severely stunted growth
ref.
Stunting (failure to grow) in early infancy is considered in some cases
to be a continuation of intrauterine growth retardation, which is prevalent
in underprivileged African communities. It is possible that children with
noma are victims of intrauterine growth retardation, which impairs development
of immune function. This hypothesis would suggest that there is a close
pathogenic similarity between noma in children and the histopathologically
similar noma neonatorum
Symptoms & signs :
patients with acute noma usually present with malodorous breath, fluctuating
fever (temperatures of 101 to 105°F [38 to 41°C]), marked anemia,
a high white-cell count, severe growth failure, and other manifestations
of malnutrition and poor general health. The lesion, usually unilateral,
is often well established by the time medical help is sought. Noma generally
starts as a gingival ulceration that can be treated easily at the early
stage with local disinfection, antibiotics, and nutritional rehabilitation.
If the ulcer is left untreated, it progresses rapidly to involve the cheek
or lip; swelling is often the earliest externally visible sign of disease.
The swelling increases, and within days, a blackish furrow appears where
intraoral tissue is being lost. The lesion finally establishes itself with
a well-demarcated perimeter surrounding a blackened necrotic center. Sequestration
of exposed bone and teeth occurs rapidly after the separation of the soft-tissue
slough. A hole remains after the scab is removed.
Prognosis : the sequelae
depend on the anatomical sites affected, the extent and severity of tissue
destruction, and the stage of development of the oral tissues. Survivors
of acute noma often have severe disfigurement and functional impairment.
They may therefore be shunned by society, and although reconstructive surgery
is possible, it rarely restores normal facial appearance
ref.
Noma neonatorum,
which is considered a distinct entity, affects newborns and resembles noma
in older children. Preterm birth and severe
intrauterine
growth retardation
are considered important predisposing factors.
Necrotizing diseases of the oral tissues associated
with HIV infection may be confused with early signs of noma, so serologic
testing for HIV should be performed when noma is suspected.
Therapy : management of
acute noma requires correction of dehydration and electrolyte imbalance,
treatment of associated diseases (such as malaria and measles), testing
for
HIV-1
infection and appropriate referral, the administration of antibiotics (penicillin
and metronidazole are generally effective), local wound care with antiseptics,
and removal of any remaining tissue slough and sequestra. No major surgery
is performed until the acute stage has been controlled. Recommendations
for the prevention of noma include the inculcation of good nutritional
practices, the promotion of exclusive breast-feeding during the first three
to six months of life, immunization against endemic communicable diseases,
proper oral hygiene practices, the segregation of livestock from human
living areas, and education about noma. Yet it is clear that the elimination
of the root causes would require improvement in living conditions through
the eradication of poverty
ref.
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