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Pemphigus
Pemphigus is a rare group of
blistering autoimmune diseases that
affect the skin and mucous
membranes.
external resources
Types
• There are three types of pemphigus which
vary in severity: pemphigus vulgaris,
pemphigus foliaceus, and paraneoplastic
pemphigus.
• The most common form of the disorder is
pemphigus vulgaris (PV - ICD-10 L10.0). It
occurs when antibodies attack Desmoglein 3.
Sores often originate in the mouth, making
eating difficult and uncomfortable. Although
pemphigus vulgaris may occur at any age, it is
most common among people between the ages
of 40 and 60. It is more frequent among
Ashkenazi Jews. Rarely, it is associated with
myasthenia gravis. Nail disease may be the only
finding and has prognostic value in
management.
Pemphigus foliaceus
• Pemphigus foliaceus (PF) is the least
severe of the three varieties. Desmoglein
1, the protein that is destroyed by the
autoantibody, is only found in the top dry
layer of the skin. PF is characterized by
crusty sores that often begin on the scalp,
and may move to the chest, back, and
face. Mouth sores do not occur. It is not as
painful as pemphigus vulgaris, and is often
mis-diagnosed as dermatitis or eczema
Paraneoplastic pemphigus
• The least common and most severe type of
pemphigus is paraneoplastic pemphigus
(PNP). This disorder is a complication of cancer,
usually lymphoma and Castleman's disease. It
may precede the diagnosis of the tumor. Painful
sores appear on the mouth, lips, and the
esophagus. In this variety of pemphigus, the
disease process often involves the lungs,
causing bronchiolitis obliterans (constrictive
bronchiolitis). Complete removal and/or cure of
the tumor may improve the skin disease, but
lung damage is generally irreversible .
Classification
• Pemphigus is a group of autoimmune
blistering diseases that may be classified
into the following types
Classification
– Pemphigus vulgaris, of which there several forms:
» Pemphigus vegetans
» Pemphigus vegetans of Hallopeau
» Pemphigus vegetans of Neumann
– Pemphigus foliaceus, of which there several forms:
» Pemphigus erythematosus
» Endemic pemphigus
– Paraneoplastic pemphigus
– IgA pemphigus, of which there several forms:
» Subcorneal pustular dermatosis
» Intraepidermal neutrophilic IgA dermatosis
Diagnosis
• Pemphigus is recognized by a
dermatologist from the appearance and
distribution of the skin lesions. It is also
commonly diagnosed by periodontists, oral
and maxillofacial surgeons (specialists
qualified in both medicine and
dentistry)and ophthalmologists (eye
doctors) as lesions can affect the eyes and
mucous membrane of the oral cavity.
Diagnosis
• Definitive diagnosis requires examination of a
skin or mucous membrane biopsy by a
dermatopathologist or oral pathologist. The skin
biopsy is taken from the edge of a blister,
prepared for histopathology and examined with
a microscope. The pathologist looks for an
intraepidermal vesicle caused by the breaking
apart of epidermal cells (acantholysis). Thus, the
superficial (upper) portion of the epidermis
sloughs off, leaving the bottom layer of cells on
the "floor" of the blister. This bottom layer of
cells is said to have a "tombstone appearance".
Treatment
• If not treated, pemphigus can be fatal due
to overwhelming infection of the sores.
The most common treatment is the
administration of oral steroids, especially
prednisone, and often in high doses. The
side effects of cortico-steroids may require
the use of so-called steroid-sparing or
adjuvant drugs. The immuno-suppressant
CellCept (Mycophenolic acid) is among
those being used
Mucosal lichen planus
• Mucosal lichen planus, or oral lichen
planus (OLP), is an inflammatory autoimmune[citation needed] disease that
affects oral mucosa, with or without the
involvement of the skin and other mucous
membranes.
Epidemiology
• OLP affects women more than men (at a
ratio of 3:2), and occurs most often in
middle-aged adults. OLP in children is
rare.
Lichen planus affecting the lower
lip.
Cause
• The cause of lichen planus is not known. It is not
contagious[1] and does not involve any known
pathogen.
OLP has been reported as a complication of
chronic hepatitis C virus infection and can be a
sign of chronic graft-versus-host disease of the
mucous membrane (and skin).
It has been suggested that OLP may respond to
stress, where lesions may present on the
mucosa (or skin) during times of stress in those
with the disease
Clinical presentation
• The reticular form is the most common presentation and manifests
as white lacy streaks on the mucosa (known as Wickham's striae) or
as smaller papules (small raised area). The lesions tend to be
bilateral and are asymptomatic. The lacy streaks may also be seen
on other parts of the mouth, including the gingiva (gums), the
tongue, palate and lips.
• The bullous form presents as fluid-filled vesicles which project from
the surface.
• The erosive form presents with erythematous (red) areas that are
ulcerated and uncomfortable. The erosion of the thin epithelium may
occur in multiple areas of the mouth, or in one area, such as the
gums, where they resemble desquamative gingivitis. Wickham's
striae may also be seen near these ulcerated areas. This form may
undergo malignant transformation.
Histo-pathological appearance
• The microscopic appearance of lichen
planus is pathognomonic for the condition
• Hyperparakeratosis with thickening of the
granular cell layer
• Development of a "saw-tooth" appearance
of the rete pegs
• Degeneration of the basal cell layer
• Infiltration of inflammatory cells into the
subepithelial layer of connective tissue
Micrograph of lichen planus. H&E
stain
Differential Diagnosis
•
•
•
•
•
•
•
•
The clinical presentation of OLP may also resemble other conditions,
including:
Lichenoid drug reaction. This entity is identical to OLP both clinically and
histologically. However, lichenoid lesions may be single (in comparison to
the usual bilateral appearance of OLP) with proximity to amalgam (metal
alloys) dental restoration.
Other oral vesiculo-ulcerative conditions such as Pemphigus vulgaris and
Benign mucous membrane pemphigoid
Discoid lupus erythematosus
Chronic ulcerative stomatitis
Frictional keratosis and Morsicatio buccarum (chronic cheek biting)
Oral leukoplakia
Chronic graft-versus-host-disease may manifest as lichenoid reaction. This
type of lichenoid lesions have a higher risk of malignant transformation to
oral squamous cell carcinoma in comparison to the classical oral lichen
planus. Graft-versus-host-disease-associated oral cancer may have more
aggressive behavior with poorer prognosis, when compared to oral cancer
in non-hematopoietic stem cell transplantation patients.
Treatment
•
•
•
•
•
•
•
•
•
Medicines used to treat lichen planus include:
Oral and topical steroids.
Oral retinoids
immunosuppressant medications
hydroxychloroquine
tacrolimus
dapsone
Aloe vera[3]
Pusley Portulaca oleracea
Lichen planus
• Lichen planus is a chronic mucocutaneous
disease that affects the skin, tongue, and oral
mucosa. The disease presents itself in the form
of papules, lesions, or rashes. Lichen planus
does not involve lichens, the fungus/algae
symbionts that often grow on tree trunks; the
name refers to the dry and undulating, "lichenlike" appearance of affected skin. It is
sometimes associated with certain medications
and diseases, but is basically of unknown cause
Lichen Planus
Classification
• Configuration
Annular lichen planus
Linear lichen planus
Morphology of lesion
Hypertrophic lichen planus
Atrophic lichen planus
Vesiculobullous lichen planus
Ulcerative lichen planus
Follicular lichen planus
Actinic lichen planus
Lichen planus pigmentosus
Classification
• Site of involvement
– Lichen planus of the palms and soles (Palmoplantar lichen
planus)
– Mucosal lichen planus
– Lichen planus of the nails
– Lichen planus of the scalp ( leading to cicatricial alopecia)
– Inverse lichen planus
• Special forms
–
–
–
–
–
–
–
Drug-induced lichen planus
Lupus erythematosus-lichen planus overlap syndrome
Lichen planus pemphigoides
Keratosis lichenoides chronica
Lichenoid reaction of graft-versus-host disease
Lichenoid keratosis
Lichenoid dermatitis
Signs and symptoms
• The typical rash of lichen planus is welldescribed by the "5 Ps": well-defined pruritic,
planar, purple, polygonal papules. The
commonly affected sites are near the wrist and
the ankle. The rash tends to heal with prominent
blue-black or brownish discoloration that persists
for a long time. Besides the typical lesions, many
morphological varieties of the rash may occur.
The presence of cutaneous lesions is not
constant and may wax and wane over time. Oral
lesions tend to last far longer than cutaneous
lichen planus lesions.
Lichen Planus
Oral lichen planus (OLP) may
present in one of three forms.
• The reticular form is the most common
presentation and manifests as white lacy streaks
on the mucosa (known as Wickham's striae) or
as smaller papules (small raised area). The
lesions tend to be bilateral and are
asymptomatic. The lacy streaks may also be
seen on other parts of the mouth, including the
gingiva (gums), the tongue, palate and lips.
• The bullous form presents as fluid-filled vesicles
which project from the surface.
Thirth form
• The erosive forms (Atrophic LP & Ulcerative LP) present
with erythematous (red) areas that are ulcerated and
uncomfortable. The erosion of the thin epithelium may
occur in multiple areas of the mouth (more prominent on
the posterior buccal mucosa), or in one area, such as the
gums, where they resemble desquamative gingivitis.
Wickham's striae may also be seen near these ulcerated
areas. This form may undergo malignant transformation,
although this is controversial. The malignant
transformation rate is thought to be less than 1%,
however it has been reported to be as high as 5%.[3] For
any persistent oral lesion of erosive lichen planus that
does not respond to topical corticosteroids, a biopsy is
recommended to rule out precancerous (premalignant)
change or malignant transformation.
Cause
• The cause of lichen planus is not known. It is not
contagious and does not involve any known
pathogen. Some lichen planus-type rashes
(known as lichenoid reactions) occur as allergic
reactions to medications for high blood pressure,
heart disease and arthritis, in such cases termed
drug-induced lichenoid reactions. These
lichenoid reactions are referred to as lichenoid
mucositis (of the mucosa) or dermatitis (of the
skin).
Treatment
•
•
•
•
•
•
•
•
•
•
•
Medicines used to treat lichen planus include:
Oral and topical steroids.
Oral retinoids
immunosuppressant medications
hydroxychloroquine
tacrolimus
dapsone
Non-drug treatments:
UVB NarrowBand Phototherapy
Aloe vera
Purslane
Lupus erythematosus
• Lupus erythematosus is a category for a
collection of diseases with similar
underlying problems with immunity
autoimmune disease
Symptoms
• Symptoms of these diseases can affect
many different body systems, including
joints, skin, kidneys, blood cells, heart, and
lungs
Four main types of lupus exist
• systemic lupus erythematosus, discoid
lupus erythematosus, drug-induced lupus
erythematosus, and neonatal lupus
erythematosus.
Systemic lupus erythematosus
• Systemic lupus erythematosus often
abbreviated to SLE or lupus, is a systemic
autoimmune disease (or autoimmune connective
tissue disease) that can affect any part of the
body. As occurs in other autoimmune diseases,
the immune system attacks the body's cells and
tissue, resulting in inflammation and tissue
damage.It is a Type III hypersensitivity reaction
caused by antibody-immune complex formation.
Systemic lupus erythematosus
Signs and symptoms
• SLE is one of several diseases known as
"the great imitators" because it often
mimics or is mistaken for other illnesses.
SLE is a classical item in differential
diagnosis, because SLE symptoms vary
widely and come and go unpredictably.
Diagnosis can thus be elusive, with some
people suffering unexplained symptoms of
untreated SLE for years.
Lupus erythematosus
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).
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
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 with antiphospholipid
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 anticoagulantpositive". 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 (LibmanSacks 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.
Laboratory tests
• 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
Diagnostic criteria
• Some physicians make a diagnosis on the
basis of the American College of
Rheumatology (ACR) classification
criteria. The criteria, however, were
established mainly for use in scientific
research including use in randomized
controlled trials which require higher
confidence levels, so some people with
SLE may not pass the full criteria
Treatment
• 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 the last medication
approved by the FDA for lupus in 1955.[61] 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
Discoid lupus erythematosus
DLE
• Discoid lupus erythematosus (DLE) is a
chronic skin condition of sores with
inflammation and scarring favoring the
face, ears, and scalp and at times on other
body areas. These lesions develop as a
red, inflamed patch with a scaling and
crusty appearance. The center areas may
appear lighter in color with a rim darker
than the normal skin.
Localized
• Localized discoid lupus erythematosus
typically presents with skin lesions
localized above the neck, with favored
sites being the scalp, bridge of nose,
cheeks, lower lip, and ears
Lupus erythematosus
• Lupus erythematosus, also called
Lupus, is a disease. It is chronic, which
means it does not go away. The Immune
system is made up of white blood cells in
your body that fight off disease.
A facial rash, as is common for
Lupus erythematosus
Mucosal lichen planus
• Mucosal lichen planus, or oral lichen
planus (OLP), is an inflammatory autoimmune[citation needed] disease that
affects oral mucosa, with or without the
involvement of the skin and other mucous
membranes.
Epidemiology
• OLP affects women more than men and
occurs most often in middle-aged adults.
OLP in children is rare.
Cause
• The cause of lichen planus is not known. It is not
contagious and does not involve any known
pathogen.
OLP has been reported as a complication of
chronic hepatitis C virus infection and can be a
sign of chronic graft-versus-host disease of the
mucous membrane (and skin).
It has been suggested that OLP may respond to
stress, where lesions may present on the
mucosa (or skin) during times of stress in those
with the disease.
Clinical presentation
• OLP may present in one of three forms.
• The reticular form is the most common presentation and manifests
as white lacy streaks on the mucosa (known as Wickham's striae) or
as smaller papules (small raised area). The lesions tend to be
bilateral and are asymptomatic. The lacy streaks may also be seen
on other parts of the mouth, including the gingiva (gums), the
tongue, palate and lips.
• The bullous form presents as fluid-filled vesicles which project from
the surface.
• The erosive form presents with erythematous (red) areas that are
ulcerated and uncomfortable. The erosion of the thin epithelium may
occur in multiple areas of the mouth, or in one area, such as the
gums, where they resemble desquamative gingivitis. Wickham's
striae may also be seen near these ulcerated areas. This form may
undergo malignant transformation.
Differential Diagnosis
•
•
•
•
•
•
•
Lichenoid drug reaction. This entity is identical to OLP both clinically and
histologically. However, lichenoid lesions may be single (in comparison to
the usual bilateral appearance of OLP) with proximity to amalgam (metal
alloys) dental restoration.
Other oral vesiculo-ulcerative conditions such as Pemphigus vulgaris and
Benign mucous membrane pemphigoid
Discoid lupus erythematosus
Chronic ulcerative stomatitis
Frictional keratosis and Morsicatio buccarum (chronic cheek biting)
Oral leukoplakia
Chronic graft-versus-host-disease may manifest as lichenoid reaction. This
type of lichenoid lesions have a higher risk of malignant transformation to
oral squamous cell carcinoma in comparison to the classical oral lichen
planus. Graft-versus-host-disease-associated oral cancer may have more
aggressive behavior with poorer prognosis, when compared to oral cancer
in non-hematopoietic stem cell transplantation patients
Treatment
• Care of OLP is within the scope of Oral medicine
speciality. It is generally accepted that OLP (as
well as other mucous membrane lichen planus,
such as genital) is more difficult to manage than
skin lichen planus.
• Currently there is no cure for lichen planus but
there are certain types of medicines used to
reduce the effects of the inflammation. Lichen
planus may go into a dormant state after
treatment. There are also reports that lichen
planus can flare up years after it is considered
cured.
Medicines used to treat lichen
planus include:
•
•
•
•
•
•
•
•
Oral and topical steroids.
Oral retinoids
immunosuppressant medications
hydroxychloroquine
tacrolimus
dapsone
Aloe vera[3]
Pusley Portulaca oleracea