120 Questions
What is the term used to describe a peculiar condition characterized by intense erythema, desquamation, and ulceration of the free and attached gingiva?
Chronic Gingivitis
In what percentage of desquamative gingivitis cases is the condition localized to the gingiva?
Approximately 50%
At what age may desquamative gingivitis occur as early as?
Puberty
What was initially suspected as the cause of chronic desquamative gingivitis?
Hormonal Derangement
What should a dentist be able to do with regards to referring a patient with desquamative gingivitis?
Perform a biopsy to confirm the diagnosis
What range of complaints do patients with chronic desquamative gingivitis have, according to the text?
Mild burning sensation to intense pain
What is the most common dermatologic cause of desquamative gingivitis?
Lichen planus
Which of the following autoimmune conditions can clinically manifest as desquamative gingivitis?
All of the above
Which type of biopsy is considered the best alternative for evaluating desquamative gingivitis?
Incisional biopsy
What is the role of direct immunofluorescence in diagnosing desquamative gingivitis?
To observe the fluorescent signal in the epithelium, basement membrane, or connective tissue
In which scenario does the patient require immediate referral to a dermatologist for further evaluation and treatment?
Pemphigus vulgaris
What is the estimated prevalence of oral lichen planus in the population?
0.1% to 1%
Which type of oral lichen planus lesions are asymptomatic, bilateral, and consist of interlacing white lines on the posterior region of the buccal mucosa?
Reticular lesions
What feature is associated with coexisting candidiasis in reticular lesions of oral lichen planus?
Erythematous background
In oral lichen planus, what do atrophic lesions of the gingival tissues result in?
Erythema confined to the gingiva
What is the characteristic feature revealed by direct immunofluorescence of oral lichen planus biopsy specimens?
Linear fibrillar deposits of fibrin in the basement membrane zone
What is the primary treatment for bullous pemphigoid, an autoimmune subepidermal bullous disease?
Systemic prednisone
Which term is preferred for bullous pemphigoid when the disease is nonscarring and mainly affects the skin?
Bullous pemphigoid
Which therapy is used for patients not responsive to corticosteroids?
Rituximab
What is the primary treatment for Linear Immunoglobulin A Disease (LAD)?
Sulfones and dapsone
Which condition usually develops in young adults between the ages of 20 and 30 years, and has a slight predilection for men?
Dermatitis Herpetiformis
Which medication is aimed at the removal of desmoglein-reactive autoantibodies in the treatment of pemphigus vulgaris?
Rituximab
What is the characteristic presentation of Linear Immunoglobulin A Disease (LAD) on the skin?
Pruritic vesiculobullous rash with annular presentation surrounded by a peripheral rim of blisters
What is the first-line pharmacologic therapy for chronic ulcerative stomatitis?
Topical steroids and topical tetracycline
Which of the following is the main immunoreactant found in biopsy tests by direct immunofluorescence in mucous membrane pemphigoid?
IgG and C3
What is the most characteristic feature of oral involvement in cicatricial pemphigoid?
Desquamative gingivitis with areas of erythema, desquamation, ulceration, and vesiculation
Which gene codes for pemphigus vulgaris?
DSG3
Which of the following is the main therapy for pemphigus vulgaris?
Systemic corticosteroids
What is the worldwide incidence of pemphigus vulgaris per year per 100,000 individuals?
0.5 to 1 case
Which of the following is associated with elevated levels of desmoglein 1 (Dsg1) autoantibodies?
Severity of cutaneous disease
Which autoimmune disease is characterized by three different clinical presentations?
Systemic Lupus Erythematosus
What is the classic cutaneous lesion characterized by the presence of a rash on the malar area with a butterfly distribution?
Systemic Lupus Erythematosus
What is the hallmark of erythema multiforme?
Central clearing
What type of lupus erythematosus usually has no systemic signs or symptoms?
Chronic Cutaneous Lupus Erythematosus
What type of lupus erythematosus presents cutaneous lesions similar to DLE but lacks the development of scarring and atrophy?
Subacute Cutaneous Lupus Erythematosus
What autoimmune disease has anti-endomysial and gliadin antibodies in almost 80% of patients?
Dermatitis Herpetiformis
Which of the following conditions is treated with a gluten-free diet?
Dermatitis Herpetiformis
What is believed to be the genesis of the mucocutaneous lesions in erythema multiforme?
Immune complex vasculitis
What is an underdiagnosed type of erythema multiforme?
The oral form
What is the drug of choice for patients with bullous or ulcerative lesions and severe symptoms of erythema multiforme?
Corticosteroids
What term was coined in 1932 to describe a peculiar condition characterized by intense erythema, desquamation, and ulceration of the free and attached gingiva?
Chronic desquamative gingivitis
What percentage of desquamative gingivitis cases are localized to the gingiva?
50%
What was initially suspected as the cause of chronic desquamative gingivitis?
Hormonal derangement
What is the characteristic feature of oral involvement in cicatricial pemphigoid?
Development of scarring and atrophy
What is the estimated prevalence of oral lichen planus in the population?
1-2%
What type of lupus erythematosus presents cutaneous lesions similar to discoid lupus erythematosus (DLE) but lacks the development of scarring and atrophy?
Subacute cutaneous lupus erythematosus (SCLE)
What are the typical reticular lesions of oral lichen planus composed of, and where are they located?
Interlacing white lines on the posterior region of the buccal mucosa
What is the primary treatment for bullous pemphigoid, an autoimmune subepidermal bullous disease?
Moderate dose of systemic prednisone
What is the characteristic feature revealed by direct immunofluorescence of oral lichen planus biopsy specimens?
Linear fibrillar ('shaggy') deposits of fibrin in the basement membrane zone
What are the distinctive patterns of gingival lesions that may occur in patients with oral lichen planus?
Keratotic lesions, erosive or ulcerative lesions, vesicular or bullous lesions, atrophic lesions
What are the clinical findings that are probably the best cognitive element to use to discriminate between bullous pemphigoid and mucous membrane pemphigoid?
Clinical findings
What is the term preferred for bullous pemphigoid when the disease is nonscarring and mainly affects the skin?
Bullous pemphigoid
What is the most common antigenic determinant associated with cicatricial pemphigoid?
BP2
In patients with pemphigus vulgaris, what do high levels of desmoglein 3 (Dsg3) autoantibodies correlate with?
severity of oral disease
What are the main immunoreactants found in biopsy tests by direct immunofluorescence in mucous membrane pemphigoid?
IgG and C3
What is the primary therapy for pemphigus vulgaris?
systemic corticosteroids
What is the term used to describe the characteristic intraepithelial clefting at the basal–spinous cell layers and interface with acantholysis seen in pemphigus vulgaris?
intercellular and intraepithelial signal
What is the estimated worldwide incidence of pemphigus vulgaris per year per 100,000 individuals?
0.1 to 0.5 cases
What is the suggested approach to establish an accurate final diagnosis of desquamative gingivitis?
A series of laboratory procedures should be used
What clinical maneuver offers insight into the plausibility of the presence of a vesiculobullous disorder?
Nikolsky’s sign
What type of biopsy is the best alternative to begin the microscopic and immunologic evaluation given the extent and number of lesions present in an individual?
Incisional biopsy
What is the term used to describe a peculiar condition characterized by intense erythema, desquamation, and ulceration of the free and attached gingiva?
Chronic ulcerative stomatitis
In what percentage of desquamative gingivitis cases does desquamative gingivitis have a dermatologic genesis?
Approximately 75%
What is the classic example of a condition that requires collaboration between a dentist and another health care provider for evaluation and treatment?
Cicatricial pemphigoid
What is the primary treatment for severe cases of chronic ulcerative stomatitis?
high dose of systemic corticosteroids
What is the primary treatment for Linear Immunoglobulin A Disease (LAD), and what can be added if the initial response is inadequate?
combination of sulfones and dapsone, small amounts of prednisone (10 mg/day to 30 mg/day)
What is the main therapy for pemphigus vulgaris, and what is currently being evaluated as an adjunct to treat it?
high dose of systemic corticosteroids, Rituximab
What is the characteristic presentation of chronic ulcerative stomatitis on the oral mucosa?
painful solitary small blisters and erosions with surrounding erythema
What is the characteristic presentation of Linear Immunoglobulin A Disease (LAD) on the skin?
pruritic vesiculobullous rash, characteristic plaques or crops with an annular presentation surrounded by a peripheral rim of blisters
What is the primary treatment for dermatitis herpetiformis, and what is it believed to be a cutaneous manifestation of?
gluten-free diet, celiac disease
What is the primary treatment for dermatitis herpetiformis?
gluten-free diet
What are the three different clinical presentations of lupus erythematosus?
systemic, chronic cutaneous, and subacute cutaneous
What is the hallmark of erythema multiforme?
Target or “iris’ lesions with central clearing
What is the term used to describe the skin lesions in chronic cutaneous lupus erythematosus?
discoid lupus erythematosus (DLE)
What is the worldwide incidence of pemphigus vulgaris per year per 100,000 individuals?
0.7 to 3.4
What is the local reaction from the use of a medicament in the oral cavity referred to as?
stomatitis venenata or contact stomatitis
What is the characteristic feature of drug eruptions in the oral cavity?
multiform, vesicular and bullous lesions
What are the main immunoreactants found in biopsy tests by direct immunofluorescence in mucous membrane pemphigoid?
IgG, IgA, and C3
What is the characteristic feature revealed by direct immunofluorescence of oral lichen planus biopsy specimens?
negative
What is the estimated prevalence of erythema multiforme in the population?
rarely seen in children (≤20%)
Desquamative gingivitis is not a specific disease entity but rather a gingival response associated with a variety of ______
conditions
Cicatricial pemphigoid and lichen planus account for more than 95% of the dermatologic ______
cases
Other conditions that may clinically manifest as desquamative gingivitis include bullous pemphigoid, pemphigus vulgaris, linear immunoglobulin A [IgA] disease, dermatitis herpetiformis, lupus erythematosus, and chronic ulcerative ______
stomatitis
Desquamative gingivitis is only a clinical term and not a diagnosis per ______
se
Given the extent and number of lesions that may be present in an individual, an incisional biopsy is the best alternative to use to begin the microscopic and ______ evaluation
immunologic
After the diagnosis is established, the dentist must choose the optimum management for the patient in accordance with three factors: the practitioner’s experience, the systemic impact of the disease, and the systemic complications of the ______
medications
The typical reticular lesions of oral lichen planus are asymptomatic and bilateral, and they consist of interlacing white lines on the posterior region of the buccal ______.
mucosa
Approximately 7% to 10% of patients with oral lichen planus have lesions restricted to the ______ tissue.
gingival
Bullous pemphigoid is a chronic, autoimmune, subepidermal bullous disease with tense cutaneous ______ that rupture and become flaccid.
bullae
The primary treatment for bullous pemphigoid is a moderate dose of systemic ______.
prednisone
Mucous membrane pemphigoid involves the oral cavity, the conjunctiva, and the mucosa of the nose, vagina, rectum, esophagus, and ______.
urethra
In about 20% of patients, the skin may also be involved in mucous membrane pemphigoid, which is also known as ______ pemphigoid.
cicatricial
Prof. Dr. TOLGA TOZUM YAKINDOĞU ÜNİVERSİTESİ DİŞHEKİMLİĞİ FAKÜLTESİ Learning outcomes: 1- Will be able to describes diseases and conditions that are seen in with ______ gingivitis.
desquamative
Patients may be asymptomatic; when symptomatic, however, their complaints range from a mild burning sensation to an intense ______.
pain
Approximately 50% of desquamative gingivitis cases are localized to the ______, although patients can have involvement of the gingiva plus other intraoral and even extraoral sites.
gingiva
Although first recognized and reported in 1894,157 the term chronic desquamative gingivitis was coined in 1932 by Prinz122 to describe a peculiar condition characterized by intense erythema, desquamation, and ulceration of the free and attached ______.
gingiva
Because most cases were diagnosed in women during the fourth and fifth decades of life (although desquamative gingivitis may occur as early as puberty or as late as the seventh or eighth decade), a hormonal ______ was suspected.
derangement
Initially, the cause of this condition was unclear, with a variety of ______ suggested.
possibilities
Pemphigus vulgaris is a potentially lethal chronic condition with a 10% mortality rate and a worldwide incidence of 0.1 to 0.5 cases per year per 100,000 individuals. A predilection in women, usually after the fourth decade of life, has been observed. However, pemphigus vulgaris has also been reported in unusually young children and even in ______.
newborns
The epidermal and mucous membrane blisters occur when the cell-to-cell adhesion structures are damaged by the action of circulating autoantibodies and by the in vivo binding of these autoantibodies to the pemphigus vulgaris antigens, which are cell-surface glycoproteins that are present in keratinocytes. These pemphigus vulgaris glycoproteins are members of the desmoglein (DSG) subfamily of the cadherin superfamily of cell–cell adhesion molecules, which are present in desmosomes. Whereas high levels of desmoglein 3 (Dsg3) autoantibodies correlate with the severity of oral disease in patients with pemphigus vulgaris, elevated levels of desmoglein 1 (Dsg1) autoantibodies are associated with severity of ______ disease.
cutaneous
The main therapy for pemphigus vulgaris is systemic corticosteroids with or without the addition of other immunosuppressive agents. If the patient responds well to corticosteroids, the dosage can be gradually reduced, but a low maintenance dosage is usually necessary to prevent or minimize the recurrence of ______.
lesions
Oral lesions can range from small vesicles to large bullae. When the bullae rupture, they leave extensive areas of ______. Virtually any region of the oral cavity can be involved, but multiple lesions often develop at sites of irritation or trauma.
ulceration
The presence of autoantibodies can be demonstrated in the oral mucosa of patients with oral pemphigus with the use of immunofluorescence techniques. For direct immunofluorescence, perilesional unfixed frozen sections are incubated with fluorescein-labeled human anti-IgG. With indirect immunofluorescence, unfixed frozen sections of oral or esophageal mucosa from an animal such as a monkey are first incubated with the patient’s serum to allow for the attachment of any serum antibodies to the mucosal tissue. The tissue is then incubated with fluorescein labeled antihuman IgG serum. Differential Diagnosis. The oral lesions of pemphigus vulgaris may be similar to those seen with ______ multiforme.
erythema
The main immunoreactants found in biopsy tests by direct immunofluorescence in mucous membrane pemphigoid are IgG and ______, which are confined to the basement membrane.
C3
In patients who are not responsive to corticosteroids or who gradually adapt to them, “steroid-sparing” therapies are used; these consist of combinations of steroids plus other medications such as ______, cyclophosphamide, cyclosporine, dapsone, gold, methotrexate
azathioprine
Rituximab is an anti-CD20 monoclonal antibody that is aimed at the removal of desmoglein-reactive autoantibodies and that is currently being evaluated as an adjunct to treat ______
pemphigus vulgaris
Chronic Ulcerative Stomatitis clinically presents with chronic oral ulcerations and has a predilection for women during their ______ decade of life
fourth
Painful solitary small blisters and erosions with surrounding erythema are present mainly on the gingiva and the lateral border of the tongue. Because of the magnitude and clinical features of the gingival lesions, a diagnosis of ______ gingivitis is considered
desquamative
Linear deposits of IgA are observed at the epithelial tissue–connective tissue interface. They differ from the granular pattern that is observed with ______ herpetiformis
dermatitis
Approximately 25% of patients with celiac disease have ______
dermatitis herpetiformis
A gluten-free diet is essential for the treatment of celiac disease and dermatitis herpetiformis. Oral ______ is usually needed for patients with newly detected dermatitis herpetiformis to alleviate symptoms promptly.
dapsone
Systemic ______ erythematosus (SLE) is a severe disease with a 10:1 predilection for women as compared with men. SLE affects vital organs such as the kidneys and heart as well as the skin and mucosa.
lupus
Chronic cutaneous lupus erythematosus usually has no systemic signs or symptoms, with ______ being limited to the skin or the mucosal surfaces. The skin ______ are referred to as discoid lupus erythematosus (DLE). DLE merely describes the chronic scarring, atrophy-producing lesion that may develop into hyperpigmentation or hypopigmentation of the healing area
lesions
Subacute cutaneous lupus erythematosus present cutaneous lesions that are similar to DLE but that lack the development of scarring and atrophy. In addition, arthritis and arthralgia, low- grade fever, malaise, and myalgia may present in up to 50% of ______ with subacute cutaneous lupus erythematosus.
patients
Erythema multiforme is an acute bullous and macular inflammatory mucocutaneous disease that affects mainly young ______ between the ages of 20 and 40 years; it is rarely seen in children (≤20%).
adults
The genesis of the mucocutaneous lesions is believed to reside in the development of immune complex ______. This is followed by complement fixation that leads to the leukocytoclastic destruction of vascular walls and small vessel occlusion.
vasculitis
There is no specific treatment for erythema multiforme. For mild symptoms, systemic and local antihistamines together with topical anesthetics and the debridement of lesions with an ______ agent are adequate.
oxygenating
An increase in the incidence of skin and oral manifestations of ______ to drugs has been noted since the advent of sulfonamides, barbiturates, and various antibiotics. The cutaneous and oral lesions are attributed to the drug acting as an allergen that sensitizes the tissues.
hypersensitivity
Eruptions in the oral cavity that result from sensitivity to drugs that have been taken by mouth or parenterally are called stomatitis ______osa. The local reaction from the use of a ______ in the oral cavity (e.g., stomatitis as a result of topical penicillin use) is referred to as stomatitis venenata or contact stomatitis.
medicament
In general, drug eruptions in the oral cavity are ______. Vesicular and bullous lesions.
multiform
Test your knowledge on the major antigenic determinants for cicatricial pemphigoid, including bullous pemphigoid 1 and 2 (BP1 and BP2), and the immune response directed against them. Explore the role of proteolytic enzymes in dissolving the basement membrane zone in the context of cicatricial pemphigoid.
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