Recommended
More Related Content
What's hot
What's hot (20)
Similar to Chronic Ulcerative Stomatitis
Similar to Chronic Ulcerative Stomatitis (20)
Recently uploaded
Recently uploaded (20)
Chronic Ulcerative Stomatitis
- 2. Learning Objectives • Preface • Etiology • Pathogenesis • Clinical features • Histopathologic features • Immunofluorescence • Comparison of the four vesiculobullous diseases
- 4. Breaking Down the Disease • Chronic long-lasting or constantly reoccurring • Ulcerative excavations of the oral mucous membranes that extends beyond the basal cell layer • Stomatitis open sores caused by a break in the oral mucous membranes that fails to heal
- 5. Etiology • Immune-mediated disorder that affects the oral mucosa Pathogenesis • Unclear pathogenic mechanism • Autoantibodies against ∆Np63α • Interrupts maintenance of the epithelial-connective tissue interface
- 6. Clinical Features • Often misdiagnosed • Elderly women, late in 6th decade • Waxes and wanes • Lesions (ulcers) in gingiva and also in the buccal mucosa and hard palate
- 7. Desquamative Gingivitis • Severity of symptoms • Half of patients have it localized to gingiva • Also characteristic of Mucous Membrane Pemphigoid, Pemphigus Vulgaris, and Erosive Lichen Planus Pemphigus Vulgaris Erosive Lichen PlanusMucous Membrane Pemphigoid Chronic Ulcerative Stomatitis
- 8. Clinical Features • Ulcers surrounded by patchy zones of erythema and streaky keratosis • Heals without scarring • Severity of oral lesions usually wax and wanes • Rare cutaneous lesions • 20% will develop concurrent lichenoid lesions
- 9. Histopathologic Features • First, biopsy the lesion • Similar to lichen planus but epithelium is more atrophic • Plasma cells and lymphocytes
- 10. Histopathologic Features • Prominent features of the epithelium • Atrophy • Liquefaction of the basal cell layer • Subepithelial clefting
- 11. Immunofluorescence • Direct Immunofluorescence (DIF) • Autoantibodies against IgG and nuclei of stratified squamous epithelial cells • Speckled pattern • Indirect Immunofluorescence • Positive for stratified epithelium- specific autoantibodies
- 12. Treatment • Topical and systemic corticosteroids • Hydroxychloroquine sulfate • Monitoring for retinopathy and periodic hematologic evaluation • Improvement or complete healing
- 13. Summary of Chronic Ulcerative Stomatitis • Often undiagnosed • Rare autoimmune disease • Autoantibodies to ∆Np63α – nuclei of stratified squamous cells • Usually localized to the gingival and buccal mucous membranes • Histopathology similar to Lichen Planus • Needs direct immunofluorescence for definitive diagnosis • Hydroxychloroquine treatment
- 14. Clinical Features Pemphigus Vulgaris Mucous Membrane Pemphigoid Lichen Planus Chronic Ulcerative Stomatitis 4 th – 6 th decade F > M, 6 th – 7 th decade F > M, 5 th – 6 th decade F >M, Late in 6 th decade
- 15. Histopathologic Features Pemphigus Vulgaris Mucous Membrane Pemphigoid Lichen Planus Chronic Ulcerative Stomatitis
- 16. Direct Immunofluorescence Pemphigus Vulgaris Mucous Membrane Pemphigoid Lichen Planus Chronic Ulcerative Stomatitis Positive, Between squamous epithelial cells Positive, Basement membrane zone Positive, Fibrinogen at Basement membrane zone Positive, Nuclei of squamous epithelial cells
- 17. Pemphigus Vulgaris Mucous Membrane Pemphigoid Lichen Planus Chronic Ulcerative Stomatitis Clinical Features 4th – 6th decade Vesicles, erosions, and ulcerations on any oral mucosal or skin surface F > M, 6th – 7th decade Primarily mucosal lesions F > M, 5th – 6th decade Oral and/or skin lesions; may or may not be erosive F >M, Late in 6th decade Gingival, buccal, and sometimes hard palate mucosal ulceration Histopathologic Features Intraepithelial clefting Subepithelial clefting Hyperkeratosis, Saw-tooth rete ridges, bandlike infiltrate of lymphocytes Infiltrate of lymphocytes and some subepithelial clefting Direct Immunofluorescence Positive, Between squamous epithelial cells Positive, Basement membrane zone Positive, Fibrinogen at Basement membrane zone Positive, Nuclei of squamous epithelial cells Indirect Immunofluorescence Positive Negative (rarely positive) Negative Positive
- 18. References • Newman, Michael, Henry Takei, Perry Klokkevold, Fermin Carranza. Carranza's Clinical Periodontology, 12th Edition. W.B. Saunders Company, 2015. VitalBook file. • Chi, Angela, Brad Neville, Douglas Damm, Carl Allen. Oral and Maxillofacial Pathology, 4th Edition. Saunders, 2016. VitalBook file.
- 19. Acknowledgements I would like to thank Dr. Anne Jones, DDS, for her guidance and example as an instructor. Additional appreciation to Daniel Chitty, Meredith Mosier, and Jacob Woods for this collaborative group effort.
- 20. Questions?
Editor's Notes
- Hi, my name is Penny Wong, I’m a second year dental student here at UT Health San Antonio, and I’m going be teaching to you about Chronic Ulcerative Stomatitis, another vesiculobullous disease that is more rare.
- So here’s our agenda for Chronic Ulcerative Stomatitis, first we are going to do a little exercise to maybe see how well you have been listening to the previous lectures Then we are going into the etiology and the pathogenesis Following that we will describe Chronic Ulcerative Stomatitis through its clinical features, its histopathologic features, and the immunofluorescence tests of it. Then to conclude everything, we will compare all the descriptive factors of the four vesiculobullous diseases we have talked about today.
- So here you have two patients. Each of them have some sort of vesiculobullous disease and they are depicted in these facial shots of their gingiva and this intraoral view of the inside of their cheek, the buccal mucosa. Looking at these and based on the information you learned earlier, are you able to tell which one of these is erosive lichen planus? Looking at just these four pictures, it is impossible to diagnose a patient, but it can help you single down to what diseases they may have and direct you, the dentist, in referring this patient. Clinically, both of these patients have an erythematous border of gingiva around their teeth and vesicles on their buccal mucosa. Pretty much the same. The patient on the left actually has Erosive Lichen Planus, and the patient on the right has Chronic Ulcerative Stomatitis. While Lichen Planus does have the characteristic clinical feature of Wickham Striae, this happens in varying intensity so there needs to be other tests to definitively diagnose Erosive Lichen Planus and Chronic Ulcerative Stomatitis. I will discuss this at the end and why it is so important. So hold on to your horses. The other patient has Chonic Ulcerative which one is erosive lichen planus, which one is chronic ulcerative stomatitis? Sometimes you cannot tell because they look very similar even thought Lichen planus will have the characteristic Wickham Striae, there are varying degrees of this condition. To have definitive diagnosis of chronic ulcerative stomatitis, there needs to be a direct immunofluoresence test that we will discuss further later.
- So what is Chronic Ulcerative Stomatitis!?! Let’s break down this long phrase Chronic means that it is long-lasting or constantly reoccuring Ulcerative means there are excavations of the oral mucous membranes that extend beyond the basal cell layer Looking at the picture of skin on the right, you have a layer of keratin at the top, which oral mucous membranes don’t have so wipe that part out of the picture, but you would still have the epithelium, depicted in underneath, the basement membrane in blue, and the connective tissue underneath. In chronic ulcerative stomatitis, the lesion will extend past that blue basement membrane and into the connective tissue. Ouch. And stomatitis means that these ulcerations will remain as open sores in the oral mucous membranes because they do not heal well.
- Why does this occur? Chronic Ulcerative Stomatitis is an immune-mediated disorder And the pathogenesis, how it exactly happens is a bit unclear But we do know the body’s own immune system produces antibodies that attack the nuclear protein, delta Np63 alpha And this interrupts the maintenance of the epithelial-connective tissue interface. Since it’s an Immune mediated disorder, that explains the chronic condition in how it will occur over a long period of time, waxing and waning, with flare ups in times of stress.
- As we saw earlier, Chronic Ulcerative Stomatitis can be easily confused with other vesiculobullous diseases and since it is rarer, it’s often misdiagnosed. As with the majority of autoimmune diseases, it occurs most commonly in women. Particularly those around 50 years old. Patients will experience a range in severity of ulcerations in their gingiva. And ulcerations can also appear in the buccal mucosa and the hard palate. One can imagine this makes it difficult to eat and cause painful gums.
- This ulceration of gingival mucosa is called desquamative gingivitis and it ails patients with Mucous membrane pemphigoid, Pemphigus vulgaris, Erosive lichen planus, and Chronic ulcerative stomatitis. Desquamative gingivitis is characterized by a generalized erythmatous border of gingiva above the necks of teeth and is due to the severe ulceration of gingival mucosa where the superficial layer of the gingival epithelium peels off. Symptoms range from a mild burning sensation of the gums to an intense pain. It’s usually contained to the gingiva, but sometimes it can happen in other intraoral sites and even extraoral ones too. Again, when you, the dentist sees desquamative gingivitis in a patient’s mouth, you should start thinking about whether it is one of these four vesiculobullous diseases and what kind of tests can you ask for to diagnose the patient’s condition.
- Continuing with the clinical features of Chronic Ulcerative Stomatitis These patients will have ulcers surrounded by patchy zones of erythema and streaky keratosis It is difficult for these ulcers to heal but they can and their severity will vary over time. It is rare for the patients to have these ulcers on their skin but a fifth of patients will develop concurrent lichenoid lesions in their mouth which can make it look exactly the same as erosive Lichen Planus.
- Now that you can clearly see that your patient has something wrong in their mouth, you should biopsy their lesion for a specimen to look at under a light microscope and have enough left for an immunofluoroscence test! Looking at this light microscope slide, at the top you have your stratified squamous epithelium, underneath that is your basement membrane, and below that, where all these cells are, is your connective tissue layer. This histopathology is very similar to lichen planus and you can see at the top the epithelium is quite atrophic In the connective tissue, there are all those dark purple circles which are plasma cells. These are present when there body detects the presence of a pathogen. This heavy infiltrate of plasma cells and lymphocytes is due to the autoantibodies of Chronic Ulcerative Stomatitis attacking the nuclei of the stratified squamous epithelial cells, thinning out the epithelial layer.
- So summarizing what happens in the oral mucosa of Chronic Ulcerative Stomatitis at the microscopic level, there is atrophy and thinning out of the stratified squamous epithelium, liquefaction of the basal cell layer underneath and subepithelial clefting. You can see this clefting where the basement membrane is lifting up off the connective tissue because there is nothing anchoring it down anymore. Also notice the empty spaces left underneath the basement membrane, this is the formation of blisters from the dying of cells.
- So alongside with looking at the specimen under a light microscope, you, as the dentist must order some tests for immunofluorescence, the most important in diagnosing vesiculobullous diseases. Using immunofluorescence, you can figure out which specific cells and their component, that antibodies are targeting and thus, figuring out the mystery of this disease. In Chronic Ulcerative Stomatitis, the autoantibodies are against IgG and the nuclei of stratified squamous epithelial cells. These things will fluoresce and will appear as a speckled pattern. This information is needed to definitively diagnose Chronic Ulcerative Stomatitis and differentiate it from the other vesiculobullous diseases You can also add on indirect immunofluorescence which will test positive for stratified epithelium-specific autoantibodies.
- So finally we get to how can we help the patient with the information we have. In mild cases, topical and systemic steroids can give the patient some relief However corticosteroids are not always effective for Chronic Ulcerative Stomatitis For better control of symptoms, the patient should be prescribed hydroxychloroquine sulfate, an antimalarial pill, that provides long-lasting remission for the patient With this drug, you must make sure the patient sees an ophthalmologist regularly to check for retinopathy and also have periodic hematologic evaluations. While steroids are prescribed to manage Lichen Planus, Hydroxychloroquine sulfate is only prescribed for Chronic Ulcerative Stomatitis in treating the vesiculobullous diseases, so it is very important that you correctly diagnose patients with the right disease so that they get the most appropriate care.
- So to summarize Chronic Ulcerative Stomatitis Often undiagnosed Rare autoimmune disease Autoantibodies to ∆Np63α – nuclei of stratified squamous cells Usually localized to the gingival and buccal mucous membranes Histopathology similar to Lichen Planus Needs direct immunofluorescence for definitive diagnosis Hydroxychloroquine treatment And it is so important to correctly diagnose your patient so they can be referred to the right specialists and get the right treatment and with that let’s go back to the very beginning,
- If you just look at the clinical features, they all occur to people in a similar age group, predominantly elderly women over men and looking at just their gingiva, it’s difficult to tell them apart. Sure in these four diseases they can happen in different places of the oral cavity and the body, but clinical features are not enough to come to a conclusion on what disease your patient has.
- Looking at the histopathologic features, you are able to tell more differences these diseases. There are more similarities between pemphigus vulgaris and Mucous membrane pemphigoid with Lichen Planus and Chronic Ulcerative Stomatitis looking kind of alike. Using this information you may be able to narrow down some of the diseases but the histopathologic features are not enough to identify exactly which vesiculobullous disease is present.
- and here, we have the four distinctly different pictures that are from the direct immunofluorescence test. They are all positive against some component of the oral mucosa and tell us exactly what disease the patient has. In Pemphigus Vulgaris there are antibodies against the glue between squamous epithelial cells In Mucous Membrane Pemphigoid there are antibodies against the basement membrane zone In Lichen Planus there are antibodies against fibrinogen at the basement membrane zone And finally in Chronic Ulcerative Stomatitis, there are antibodies against the nuclei of squamous epithelial cells
- So again I want to reiterate how important it is to look at the overall picture and the specific test of direct immunofluorescence to definitively diagnose your patient with pemphigus vulgaris, mucous membrane pemphigoid, lichen planus, or chronic ulcerative stomatitis and to get them the help they need That is part of our duty as healthcare providers.