Oral manifestation of the dermatoses with autoimmune components.
Oral lichen planus
Oral lichen planus (LIE-kun PLAY-nus) is an
inflammatory condition that affects mucous membranes inside the
mouth. Oral lichen planus may appear as white, lacy patches; red, swollen
tissues; or open sores. These lesions may cause burning, pain or other
discomfort.
Oral lichen planus can't be passed from one
person to another. The disorder occurs when the immune system mounts an attack
against cells of the oral mucous membranes. The reason for this abnormal immune
response is unknown.
Oral lichen planus is usually an ongoing
(chronic) condition. Treatments that suppress the immune system abnormalities
may improve more severe lesions and lessen pain.
People with oral lichen planus may also have
related lichen planus lesions on the skin, genitals or other parts of the body.
Causes
The cause of oral lichen planus is
unknown. The lesions that appear are the result of inflammation controlled by
specific white blood cells called T lymphocytes. Normally, these cells are
active at the site of disease or injury.
However, certain diseases, medical
conditions or other factors may act as triggers of the inflammatory disorder in
some people.
Factors that may act as triggers of oral
lichen planus:
Factors that may complicate the condition
or worsen symptoms include:
Anyone can develop oral
lichen planus, but the condition most often affects middle-aged women.
The primary signs and symptoms of oral
lichen planus are the lesions affecting the mucous membranes of the mouth.
Appearance
The lesions may appear as:
Location
These lesions may appear on the following sites:
Pain or discomfort
The red, inflamed lesions and open sores of oral lichen planus can cause a
burning sensation or pain. The white,
lacy patches alone usually don't cause discomfort, except when they appear
on the tongue.
Other signs or symptoms
Other signs or symptoms may include:
Other types of lichen
planus
Additional tests:
Corticosteroids
Corticosteroids may reduce inflammation associated with oral lichen planus. The
side effects vary depending on whether it's used as a mouthwash or ointment
applied directly to the mucous membrane (topical), taken as a pill (oral), or
administered as an injection. The potential benefit of corticosteroids needs to
be balanced with possible side effects, which include the following:
Retinoids
Retinoids are synthetic versions of vitamin A that can be applied as a topical
ointment or taken orally. The topical treatment doesn't cause the same side
effects associated with corticosteroids, but it may irritate the mucous
membranes of the mouth.
Because both topical and oral retinoids can cause birth defects,
the drug shouldn't be used by women who are pregnant or planning to become
pregnant in the near future.
Nonsteroidal ointments
In the past few years, several reports have shown the effectiveness of topical
medications, called calcineurin inhibitors, which are closely related to or
identical to oral medications used to prevent rejection of transplanted organs.
These treatments appear to be effective for the treatment of oral lichen
planus. Examples of these topical medications include tacrolimus (Protopic
ointment) and pimecrolimus (Elidel cream).
Addressing triggers
Systemic lupus erythematosus
Systemic lupus erythematosus (SLE) is a long-term autoimmune disorder that
may affect the skin, joints, kidneys, brain, and other organs.
SLE is one of several diseases known as "the great imitators" because it often mimics or is mistaken for other illnesses
Systemic lupus erythematosus (SLE) is an
autoimmune disease, which means the body's immune system mistakenly attacks
healthy tissue. This leads to long-term (chronic) inflammation.
The underlying cause of autoimmune diseases is
not fully known.
SLE is much more common
in women than men. It may occur at any age, but appears most often in people
between the ages of 10 and 50. African Americans and Asians are affected more
often than people from other races. SLE may also be caused
by certain drugs.
Symptoms vary from person to person, and may come and go.
Almost everyone with SLE has joint pain and
swelling. Some develop arthritis. Frequently affected
joints are the fingers, hands, wrists, and knees.
Other common symptoms include:
Other symptoms depend on what part of the body is affected:
Some
patients only have skin symptoms. This is called discoid lupus.
Dermatological manifestations
As many as 30% of sufferers have some dermatological
symptoms (and 65% suffer such symptoms at some point), with 30% to 50%
suffering from the classic malar rash (or butterfly rash)
associated with the disease. Some may exhibit thick, red scaly patches on the
skin (referred to as discoid lupus). Alopecia; mouth, nasal, urinary tract and vaginal ulcers,
and lesions on the skin are also possible manifestations. Tiny tears in
delicate tissue around the eyes can occur after even minimal rubbing.
Musculoskeletal
The most commonly sought medical attention is
for joint pain,
with the small joints of the hand and wrist usually affected, although all
joints are at risk. The Lupus Foundation of America estimates
more than 90 percent of those affected will experience joint and/or muscle pain
at some time during the course of their illness.Unlike rheumatoid
arthritis, lupus arthritis is less disabling and usually does not
cause severe destruction of the joints. Fewer than ten percent of people with
lupus arthritis will develop deformities of the hands and feet. SLE patients are at particular risk of developing osteoarticular tuberculosis.
A possible association between rheumatoid
arthritis and SLE has been suggested, and SLE may be
associated with an increased risk of bone
fractures in relatively young women.
Hematological
Anemia may
develop in up to 50% of cases. Low platelet and white blood
cell counts may be due to the disease or a side effect of
pharmacological treatment. People with SLE may have an association withantiphospholipid antibody syndrome (a
thrombotic disorder), wherein autoantibodies to phospholipids are present in
their serum. Abnormalities associated with antiphospholipid antibody syndrome
include a paradoxical prolonged partial thromboplastin time (which
usually occurs in hemorrhagic disorders) and a positive test for antiphospholipid
antibodies; the combination of such findings have earned the term "lupus
anticoagulant-positive". Another autoantibody finding in SLE is
the anticardiolipin antibody, which can cause
a false positive test for syphilis.
Cardiac
A person with SLE may have inflammation of various parts of the heart, such as pericarditis, myocarditis, and endocarditis.
The endocarditis of SLE is characteristically noninfective (Libman-Sacks endocarditis), and involves
either the mitral valve or
the tricuspid
valve. Atherosclerosis also tends to occur more
often and advances more rapidly than in the general population.
Pulmonary
Lung and pleura inflammation can cause pleuritis, pleural
effusion, lupus pneumonitis, chronic diffuse interstitial lung
disease, pulmonary hypertension, pulmonary
emboli, pulmonary hemorrhage, and shrinking lung
syndrome.
Renal
Painless hematuria or proteinuria may often
be the only presenting renal symptom. Acute or chronic renal impairment may
develop with lupus nephritis, leading to acute or
end-stage renal failure.
Because of early recognition and management of SLE, end-stage renal failure
occurs in less than 5% of cases.
A histological hallmark of SLE is membranous glomerulonephritis with
"wire loop" abnormalities. This finding is due to immune complex
deposition along the glomerular basement membrane, leading to a
typical granular appearance in immunofluorescence testing.
Neuropsychiatric
Neuropsychiatric syndromes
can result when SLE affects the central or peripheral nervous systems. The American College of Rheumatology defines
19 neuropsychiatric syndromes in systemic lupus erythematosus. The diagnosis of neuropsychiatric syndromes concurrent with SLE is one of
the most difficult challenges in medicine, because it can involve so many
different patterns of symptoms, some of which may be mistaken for signs of
infectious disease or stroke.
The most common neuropsychiatric disorder people with SLE have is headache,although the
existence of a specific lupus headache and the optimal approach to
headache in SLE cases remains controversial. Other common neuropsychiatric
manifestation of SLE include cognitive
dysfunction, mood disorder, cerebrovascular
disease, seizures, polyneuropathy, anxiety
disorder, and psychosis. It can rarely present with intracranial hypertension syndrome,
characterized by an elevated intracranial
pressure, papilledema, and headache with
occasional abducens nerve paresis, absence of a
space-occupying lesion or ventricular enlargement, and normalcerebrospinal
fluid chemical and hematological constituents.
More rare manifestations are acute confusional state, Guillain-Barré syndrome, aseptic
meningitis, autonomic disorder, demyelinating
syndrome, mononeuropathy (which might manifest
as mononeuritis multiplex), movement
disorder (more specifically, chorea), myasthenia
gravis, myelopathy, cranial neuropathy and plexopathy.
Neurological
Neural symptoms contribute to a significant percentage of morbidity and
mortality in patients with lupus. As a result,
the neural side of lupus is being studied in hopes of reducing morbidity and
mortality rates. The neural
manifestation of lupus is known as neuropsychiatric systematic lupus
erythematosus (NPSLE). One aspect of this disease is severe damage to the
epithelial cells of the blood–brain
barrier.
Lupus has a wide range of symptoms which span the body. The neurological
symptoms include headaches, depression, seizures, cognitive
dysfunction, mood disorder, cerebrovascular
disease, polyneuropathy, anxiety
disorder, psychosis,
and in some extreme cases, personality disorders. In certain regions, depression reportedly affects up to 60% of women
suffering from SLE.
Reproductive
Further
information: Systemic lupus erythematosus and
pregnancy
SLE causes an increased rate of fetal death in utero and spontaneous
abortion (miscarriage). The overall live-birth rate in SLE
patients has been estimated to be 72%. Pregnancy
outcome appears to be worse in SLE patients whose disease flares up during
pregnancy.
Neonatal
lupus is the occurrence of SLE symptoms in an infant born from a
mother with SLE, most commonly presenting with a rash resembling discoid lupus erythematosus, and sometimes
with systemic abnormalities such as heart block or hepatosplenomegaly.Neonatal
lupus is usually benign and self-limited.
Systemic
Fatigue in
SLE is probably multifactorial and has been related to not only disease
activity or complications such as anemia or hypothyroidism, but also to pain, depression,
poor sleep quality,
poor physical
fitness and perceived lack of social
support.
The first mechanism may arise genetically. Research
indicates SLE may have a genetic link. SLE does run in families, but no
single causal gene has been identified. Instead, multiple genes appear to
influence a person's chance of developing lupus when triggered by environmental
factors. The most important genes are located in the HLA region on chromosome 6,
where mutations may occur randomly (de novo) or may be inherited. HLA
class I, class II, and class III are associated with SLE, but only classes I
and II contribute independently to increased risk of SLE. Other genes which
contain risk variants for SLE are IRF5, PTPN22, STAT4, CDKN1A, ITGAM, BLK, TNFSF4 and BANK1.
Some
of the susceptibility genes may be population specific.
One manifestation of
SLE is abnormalities in apoptosis, a type of
programmed cell death in which aging or damaged cells are neatly disposed of as
a part of normal growth or functioning.
Antinuclear antibody (ANA) testing and anti-extractable
nuclear antigen (anti-ENA)
form the mainstay of serologic testing for SLE. Several techniques
are used to detect ANAs. Clinically the most widely used method is indirect immunofluorescence.
The pattern of fluorescence suggests the type of antibody present in the
patient's serum.
ANA screening yields positive results in many connective
tissue disorders and other autoimmune diseases, and may occur in normal
individuals. Subtypes of antinuclear antibodies include anti-Smith and
anti-double strandedDNA (dsDNA) antibodies (which are linked
to SLE) and anti-histone
antibodies (which are
linked to drug-induced lupus). Anti-dsDNA antibodies are highly specific for
SLE; they are present in 70% of cases, whereas they appear in only 0.5% of
people without SLE. The anti-dsDNA antibody titers also tend to reflect disease activity,
although not in all cases.Other ANA that may occur in SLE sufferers are anti-U1 RNP (which also appears in systemic
sclerosis), SS-A (or anti-Ro) and SS-B (or anti-La; both of which are more common in Sjögren's
syndrome). SS-A and SS-B confer a specific risk for heart conduction
block in neonatal lupus.
Other tests routinely performed in suspected SLE are complement
system levels (low
levels suggest consumption by the immune system), electrolytes and renal
function (disturbed if
the kidney is involved), liver enzymes,
and complete
blood count.
The lupus erythematosus (LE) cell test was commonly used for
diagnosis, but it is no longer used because the LE cells are
only found in 50–75% of SLE cases, and they are also found in some people with
rheumatoid arthritis, scleroderma, and drug sensitivities. Because of this, the
LE cell test is now performed only rarely and is mostly of historical
significance.
Recursive partitioning has been used to identify more
parsimonious criteria.This analysis presented two diagnostic classification
trees:
1. Simplest
classification tree: SLE is diagnosed if a person has an immunologic disorder
(anti-DNA antibody, anti-Smith antibody, false positive syphilis test, or LE
cells) or malar rash. It has
sensitivity = 92% and specificity = 92%.
2. Full
classification tree: Uses 6 criteria. It has sensitivity = 97% and specificity
= 95%.
The treatment of SLE involves preventing flares and reducing
their severity and duration when they occur.
Treatment can include corticosteroids and anti-malarial drugs. Certain types
of lupus nephritis such as diffuse proliferative glomerulonephritis require
bouts of cytotoxic drugs. These drugs include cyclophosphamide and mycophenolate.
Hydroxychloroquine (HCQ) was approved by the FDA for
lupus in 1955. Some drugs approved for other diseases are used for SLE
'off-label'. In November 2010, an FDA advisory panel recommended approving Benlysta (belimumab)
as a treatment for the pain and flare-ups common in lupus. The drug was
approved by the FDA in March 2011.
Erythema multiforme
Erythema
multiforme (EM) is an acute, self-limited, and sometimes recurring skin
condition that is considered to be a type IV hypersensitivity reaction
associated with certain infections, medications, and other various triggers.
Symptoms:
•
Fever
•
General ill feeling
•
Itching of the skin
•
Joint aches
•
Multiple skin lesions:
– Start
quickly and may return
– May
spread
– May
appear as a nodule, papule, or macule and may look
like hives
– Central
sore surrounded by pale red rings, also called a "target",
"iris", or "bulls-eye"
– May
have vesicles and blisters of various sizes (bullae)
– Located
on the upper body, legs, arms, palms, hands, or feet
– May
involve the face or lips
– Usually
even on both sides (symmetrical)
•
Bloodshot eyes
•
Dry eyes
•
Eye burning, itching, and discharge
•
Eye pain
•
Mouth sores
•
Vision problems
History:
•
In EM, there may be no prodrome or a
mild upper respiratory tract infection.
•
The rash starts abruptly, usually within
3 days. It starts on the extremities, being symmetrical and spreading
centrally.
•
Half of children with the rash have
recent herpes labialis.
•
It usually precedes the erythema
multiforme by 3 to 14 days but it can sometimes be present at the onset.
Examination:
•
The iris or target lesion is the
classical feature of the disease.
•
Initially there is a dull red flat spot
or wheal that enlarges slightly up to 2 cm over 24 to 48 hours.
•
In the middle, a small bump, vesicle, or
bulla develops, flattens, and then may clear. The intermediate ring forms and
becomes raised, pale, and swollen. The periphery slowly becomes purple and
forms a concentric lesion, resembling a target. Some lesions are atypical
targets with only 2 concentric rings.
•
The Koebner phenomenon may occur. This
is where a lesion occurs along the line of trauma and it is typical of
psoriasis and lichen planus.
•
Lesions appear first on the extensor
surfaces of the periphery and extend centrally. The palms, neck and face are
often involved but the soles and flexures of the extremities less often.
•
There may be mucosal involvement in 70%
of patients but it tends to be mild and limited to just one mucosal surface
(for example mouth or vulva).
•
Oral lesions are most common with lips,
palate and gingiva affected. There may be red conjunctivae and tearing, but eye
involvement tends to be mild.
•
Genital involvement can produce painful
hemorrhagic bullae and erosions.
Investigations:
No specific investigations are indicated.
•
Nikolsky's sign is negative
•
A punch biopsy may be required to
confirm diagnosis.
Management:
In recurrent disease due to HSV, antiviral therapy is helpful.
•
Symptomatic treatment may include
analgesics, mouth wash and local skin care.
•
Steroid creams may be used. If the mouth
is very sore, attention may have to be given to hydration and nutrition.
•
Lubricating drops for eyes may be
required.
Stevens–Johnson
syndrome:
•
Stevens–Johnson syndrome (SJS)
and toxic epidermal necrolysis (TEN) are two forms of a
life-threatening skin condition, in which cell death causes
the epidermis to separate from the dermis
•
The syndrome is thought to be
a hypersensitivity complex that affects the skin and the mucous
membranes. Although the majority of cases are idiopathic (without a known cause), the main
class of known causes is medication, followed by infections and, rarely,
cancers.
Symptoms
•
SJS usually begins with fever, sore
throat, and fatigue, which is misdiagnosed and usually treated with
antibiotics.
•
Ulcers and other lesions begin to appear
in the mucous membranes, almost always in the mouth and lips but also in the
genital and anal regions.
•
Those in the mouth are usually extremely
painful and reduce the patient's ability to eat or drink.
•
Conjunctivitis of the eyes occurs in about 30% of
children who develop SJS.
•
A rash of round lesions about an inch
across arises on the face, trunk, arms and legs, and soles of the feet, but
usually not the scalp.
Stevens-Johnson syndrome – affection of the tongue
Lips lesions
in SJS
Treatment:
•
SJS constitutes a dermatological
emergency. All medications should be discontinued, particularly those known to
cause SJS reactions. Patients with documented mycoplasma infections
can be treated with oral macrolide or oral doxycycline.
•
Initially, treatment is similar to that
for patients with thermal burns, and continued care can only be supportive
(e.g. intravenous fluids and nasogastric or parenteral
feeding) and symptomatic (e.g.,analgesic mouth rinse for mouth
ulcer).
•
Dermatologists and surgeons tend to
disagree about whether the skin should be debrided.
•
Beyond this kind of supportive care,
there is no accepted treatment for SJS. Treatment
with corticosteroids is controversial.
•
Early retrospective studies suggested that
corticosteroids increased hospital stays and complication rates. There are no
randomized trials of corticosteroids for SJS, and it can be managed
successfully without them.
•
Intravenous immunoglobulin (IVIG) treatment has shown some
promise in reducing the length of the reaction and improving symptoms. Other
common supportive measures include the use of topical
pain anesthetics and antiseptics, maintaining a warm
environment, and intravenous analgesics. An ophthalmologist should be
consulted immediately, as SJS frequently causes the formation of scar tissue
inside the eyelids, leading to corneal vascularization, impaired vision and a
host of other ocular problems.
References:
1. Danilevskiy M.F. et al. “ Diseases of the
mucous membrane of the mouth.” - K.: "Medytsyna", 2010.
2. Bruch J.M. Clinical oral medicine and
pathology/ J.M. Bruch, N.S. Treister// London.:Humana Press, 2010
3. Cawson R. E. Cawson’s essentials of oral
pathology and oral medicine. Seventh edition/ Cawson R. E. et. al. //Elsevier
science limited, 2002.
4. Slootweg P. Dental pathology – a practical
introduction/ P.J. Slootweg// Berlin.: Springer, 2007.
5. Da Silva J.D. Oxford American Handbook of
Clinical Dentistry (Oxford American Handbooks in Medicine) / J.D. Da Silva et
al.// Oxford University Press, 2007.
.
Information was prepared by Sukhovolets I.O.