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PEMPHIGUS VULGARIS
“A CASE REPORT”
INDIAN DENTAL ACADEMY
Leader in continuing Dental Education
www.indiandentalacademy.c...
Pemphigus is derived from the Greek word
‘Pemphix’ meaning bubble or a blister .
Pemphigus describes a group of chronic
...
Pemphigus refers to a group of
autoimmune blistering diseases of
the skin and mucous membrane
characterized histologically...
www.indiandentalacademy.com
Indian Dental academy
• www.indiandentalacademy.com
• Leader continuing dental education
• Off...
• Pemphigus Vulgaris has been reported to occur
world wide, incidence varies from 0.5 to 5 cases
per 1,00,000.
(Langan SM ...
As the Oral manifestations in Pemphigus
occur much before the skin lesions , Dental
Professionals must be sufficiently fa...
CASE REPORT
www.indiandentalacademy.com
CHIEF COMPLAINT
A 48 year old male patient
reported to the our OPD for the
treatment of recurring
ulcerations in his mout...
The Patients past Medical and Drug history
were non contributory and there was no
significant Medical Problem.
Patient h...
Healed Scars of the blisters were seen on the scalp and nose
Nikolskys sign was POSITIVE
EXAMINATION
 Patient wasPatient ...
Denuded blisters of various sizes ranging from the size
0.25x0.25 cm to 0.5cmx0.5cms were seen on the lower lip,
legs and...
LESION ON THE LEG LESION ON THE LIP
LESIONS ON THE SCALP LESIONS ON THE BACK
www.indiandentalacademy.com
No eye involvement was seen.
www.indiandentalacademy.com
INTRA ORAL EXAMINATION
INSPECTION
Shallow ulcers of the size
ranging from 5cmsx3cms to
4cmsx2cms were present on
the bucc...
One hemorrhagic bulla
of the size 3cms x 2cms
was present on the buccal
mucosa opposite the 1st
molar.
DENTAL
Other part...
PALPATION
The ulcers were tender
on palpation
Tendency to bleeding
on slightest provocation
was present
The floor of th...
PROVISIONAL DIAGNOSIS: Pemphigus
Age (48 years) and Chronicity.
Presence of multiple ulcers as well as Bullous lesions
i...
DIFFERENTIAL DIAGNOSIS
Bullous Pemphigoid
Cicatricial Pemphigoid
Erythema Multiforme
Erosive Lichen Planus
Allergic S...
INVESTIGATIONS
ROUTINE BLOOD
SERUM IgG ANTIBODY TITRE
EXFOLIATIVE CYTOLOGY
INCISIONAL BIOPSY
www.indiandentalacademy.com
BLOOD INVESTIGATIONS
All blood investigations were within the
normal limits except ESR which was found
to be on a higher ...
Exfoliative Cytology
Cytosmear showed
presence of eosinophilic
rounded cells with
darkly stained
hyperchromatic nucleus
e...
Incisional BiopsyParakeratinized stratified
squamous epithelium with
intra epithelial split also
showing basal cells abut...
FINAL DIAGNOSIS
PEMPHIGUS VULGARIS
www.indiandentalacademy.com
Treatment
The patient was put on
0.5mg of Tab Betnesol TDS
X 8days Swish and Swallow
therapy
Dose was tapered on week
ba...
Betadine mouth rinses were advised to
combat secondary infection.
Supportive therapy with high dosage of
Antioxidants we...
PRE- TREATMENT 2- Week- Follow upwww.indiandentalacademy.com
PRE- TREATMENT 2 Week-Follow Upwww.indiandentalacademy.com
PRE- TREATMENT
2 Week-Follow Upwww.indiandentalacademy.com
DISCUSSION
Variants of Pemphigus are:
Pemphigus vulgaris
Pemphigus foliaceus
Pemphigus paraneoplastic
Drug Induced Pem...
Pemphigus Vulgaris is the most common form and
frequently involves the mouth.
(Weinberg et al.)
Patients with active disea...
PATHOPHYSIOLOGY
SUPRA BASILAR BULLA
Abnormal IgG Production
Binding of specific IgG
antibodies to an antigen on
the epithe...
CAUSES
The cause of Pemphigus Vulgaris remains
unknown. However, several relevant factors
have been identified.
Genetic F...
CLINICAL FEATURES
SKIN LESIONS
www.indiandentalacademy.com
Ordinary Pemphigus
Vulgaris may develop
vegetative lesions in the
skin folds forming
vegetating granulations.
This form ca...
MUCOSAL MANIFESTATIONS
Mucous membranes typically are affected first
and preceded by cutaneous lesions.
 Oral lesions ar...
Oral lesions may begin
as the classic bulla
Shallow ulcers are
seen as the bullae
break rapidly.
Erosions can be seen in...
Other mucosal
surfaces may be
involved,
including the
conjunctiva,
esophagus,
vagina, cervix,
penis, urethra
and anus.
ww...
DIF SHOWING DEPOSITION OF
IMMUNOREACTANTS IN THE INTERCELLULAR
AREAS BETWEENTHE SURFACE EPITHELIAL CELLS
PEMPHIGOIDPEMPHIG...
MANAGEMENT
The aim of treatment in Pemphigus
vulgaris is the same as in other
autoimmune bullous diseases , which is to
d...
www.indiandentalacademy.com
SYSTEMIC STEROIDS
High doses of systemic corticosteroids along
with immunosuppressive drugs such as
Azithioprine, Dapsone...
ALTERNATIVE TREATMENT TO
CORTICOSTEROIDS
DRUGS:
1) Mycophenolate Mophetil
2) Rituximab
3) Etanercept
4) Immunoglobulins
5)...
MANAGEMENT
www.indiandentalacademy.com
CONCLUSION
In this case report, we describe the management of a
patient who had previously undergone treatment ,
whose co...
BIBLIOGRAPHY
1. Mignogna et al: Oral pemphigus: long term behaviour and clinical
response to treatment with deflazacort in...
www.indiandentalacademy.com
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pemphigus vulgaris/ dental courses


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pemphigus vulgaris/ dental courses

  1. 1. PEMPHIGUS VULGARIS “A CASE REPORT” INDIAN DENTAL ACADEMY Leader in continuing Dental Education www.indiandentalacademy.com
  2. 2. Pemphigus is derived from the Greek word ‘Pemphix’ meaning bubble or a blister . Pemphigus describes a group of chronic bullous diseases, Originally named by ‘Wichman’ in 1791. www.indiandentalacademy.com
  3. 3. Pemphigus refers to a group of autoimmune blistering diseases of the skin and mucous membrane characterized histologically by intradermal blisters and immunopathologically by the finding of in vivo bound and circulating immunoglobulin G (IgG) antibody directed against the cell surface of keratinocytes. Shafers. Definition www.indiandentalacademy.com
  4. 4. www.indiandentalacademy.com Indian Dental academy • www.indiandentalacademy.com • Leader continuing dental education • Offer both online and offline dental courses
  5. 5. • Pemphigus Vulgaris has been reported to occur world wide, incidence varies from 0.5 to 5 cases per 1,00,000. (Langan SM et al).  Male to female ratio is approximately equal .  Mean age of onset is 40-70 years of age . (Turki Hamida et al) INCIDENCE www.indiandentalacademy.com
  6. 6. As the Oral manifestations in Pemphigus occur much before the skin lesions , Dental Professionals must be sufficiently familiar with Clinical manifestations for early Diagnosis and Management. www.indiandentalacademy.com
  7. 7. CASE REPORT www.indiandentalacademy.com
  8. 8. CHIEF COMPLAINT A 48 year old male patient reported to the our OPD for the treatment of recurring ulcerations in his mouth at different sites since 1 year. Burning sensation on eating spicy food . He also complained of appearance of blisters that came into existence after the oral ulcerations, involving the legs, scalp and back. www.indiandentalacademy.com
  9. 9. The Patients past Medical and Drug history were non contributory and there was no significant Medical Problem. Patient had no adverse habits .  The Vital signs were within normal limits. www.indiandentalacademy.com
  10. 10. Healed Scars of the blisters were seen on the scalp and nose Nikolskys sign was POSITIVE EXAMINATION  Patient wasPatient was moderately built and nourishedmoderately built and nourished and weighed 85 kgs.and weighed 85 kgs.  Patient wasPatient was well oriented , conscious andwell oriented , conscious and cooperativecooperative.. www.indiandentalacademy.com
  11. 11. Denuded blisters of various sizes ranging from the size 0.25x0.25 cm to 0.5cmx0.5cms were seen on the lower lip, legs and back. Central erythmatous zone with epidermal crusting at the periphery was seen on the blisters. www.indiandentalacademy.com
  12. 12. LESION ON THE LEG LESION ON THE LIP LESIONS ON THE SCALP LESIONS ON THE BACK www.indiandentalacademy.com
  13. 13. No eye involvement was seen. www.indiandentalacademy.com
  14. 14. INTRA ORAL EXAMINATION INSPECTION Shallow ulcers of the size ranging from 5cmsx3cms to 4cmsx2cms were present on the buccal mucosa of the left side, extending from premolar region till the retromolar area. The ulcers had irregular borders with mild erythema around the periphery.The surface was covered with yellowish slough. www.indiandentalacademy.com
  15. 15. One hemorrhagic bulla of the size 3cms x 2cms was present on the buccal mucosa opposite the 1st molar. DENTAL Other parts of the oral cavity were not involved. Generalized attrition was present with moderate stains and calculus www.indiandentalacademy.com
  16. 16. PALPATION The ulcers were tender on palpation Tendency to bleeding on slightest provocation was present The floor of the ulcer was covered with yellow colored slough www.indiandentalacademy.com
  17. 17. PROVISIONAL DIAGNOSIS: Pemphigus Age (48 years) and Chronicity. Presence of multiple ulcers as well as Bullous lesions involving mucocutaneous surfaces (Oral Cavity and Skin). Oral ulcers preceded cutaneous lesions. No response to earlier treatment by other physicians. www.indiandentalacademy.com
  18. 18. DIFFERENTIAL DIAGNOSIS Bullous Pemphigoid Cicatricial Pemphigoid Erythema Multiforme Erosive Lichen Planus Allergic Stomatitis Dermatitis Herpetiformis www.indiandentalacademy.com
  19. 19. INVESTIGATIONS ROUTINE BLOOD SERUM IgG ANTIBODY TITRE EXFOLIATIVE CYTOLOGY INCISIONAL BIOPSY www.indiandentalacademy.com
  20. 20. BLOOD INVESTIGATIONS All blood investigations were within the normal limits except ESR which was found to be on a higher side(24mm/hr). •The Serum IgG antibody titre of the patient was 1333mg/dl. www.indiandentalacademy.com
  21. 21. Exfoliative Cytology Cytosmear showed presence of eosinophilic rounded cells with darkly stained hyperchromatic nucleus either present in clumps or as individual cells. The acantholytic cells were interspread with numerous exfoliated buccal cells representing cells of the keratinizing layers. IMPRESSION: Acantholytic-Tzank cells probably representing Pemphigus Vulgaris. www.indiandentalacademy.com
  22. 22. Incisional BiopsyParakeratinized stratified squamous epithelium with intra epithelial split also showing basal cells abutting on the underlying connective tissue. Tzank cells were seen within the split arranged in the groups or single cells. IMPRESSION: Compatible with clinical diagnosis of Pemphigus Vulgaris. Suprabasilar Split www.indiandentalacademy.com
  23. 23. FINAL DIAGNOSIS PEMPHIGUS VULGARIS www.indiandentalacademy.com
  24. 24. Treatment The patient was put on 0.5mg of Tab Betnesol TDS X 8days Swish and Swallow therapy Dose was tapered on week basis for a period of 3 weeks. Maintenance therapy was followed. 2-Week Follow Up www.indiandentalacademy.com
  25. 25. Betadine mouth rinses were advised to combat secondary infection. Supportive therapy with high dosage of Antioxidants were also prescribed along with B-complex & Zinc supplements. Considerable improvement in the skin and oral lesions was seen after 2 weeks of therapy. Subsequent referral were made to dermatologist & physician for follow-up and further care. www.indiandentalacademy.com
  26. 26. PRE- TREATMENT 2- Week- Follow upwww.indiandentalacademy.com
  27. 27. PRE- TREATMENT 2 Week-Follow Upwww.indiandentalacademy.com
  28. 28. PRE- TREATMENT 2 Week-Follow Upwww.indiandentalacademy.com
  29. 29. DISCUSSION Variants of Pemphigus are: Pemphigus vulgaris Pemphigus foliaceus Pemphigus paraneoplastic Drug Induced Pemphigus www.indiandentalacademy.com
  30. 30. Pemphigus Vulgaris is the most common form and frequently involves the mouth. (Weinberg et al.) Patients with active disease have circulating and tissue bound autoantibodies of both the immunoglobulin G1(IgG1) and immunoglobulin G4 (IgG4) subclasses. (Scully et al) www.indiandentalacademy.com
  31. 31. PATHOPHYSIOLOGY SUPRA BASILAR BULLA Abnormal IgG Production Binding of specific IgG antibodies to an antigen on the epithelial membrane (Desmoglin-3) Epithelial cell seperation Acantholysis www.indiandentalacademy.com
  32. 32. CAUSES The cause of Pemphigus Vulgaris remains unknown. However, several relevant factors have been identified. Genetic Factor Age Disease Association Drugs www.indiandentalacademy.com
  33. 33. CLINICAL FEATURES SKIN LESIONS www.indiandentalacademy.com
  34. 34. Ordinary Pemphigus Vulgaris may develop vegetative lesions in the skin folds forming vegetating granulations. This form can be more resistant to therapy and can remain in one place for a long period of time. Vegetative Pemphigus Vulgaris: www.indiandentalacademy.com
  35. 35. MUCOSAL MANIFESTATIONS Mucous membranes typically are affected first and preceded by cutaneous lesions.  Oral lesions are the “The first to Show and Last to go” (Navaille)  Buccal mucosa  Palate  Gingiva www.indiandentalacademy.com
  36. 36. Oral lesions may begin as the classic bulla Shallow ulcers are seen as the bullae break rapidly. Erosions can be seen in any part of the oral cavity. Involve the larynx with subsequent hoarseness. www.indiandentalacademy.com
  37. 37. Other mucosal surfaces may be involved, including the conjunctiva, esophagus, vagina, cervix, penis, urethra and anus. www.indiandentalacademy.com
  38. 38. DIF SHOWING DEPOSITION OF IMMUNOREACTANTS IN THE INTERCELLULAR AREAS BETWEENTHE SURFACE EPITHELIAL CELLS PEMPHIGOIDPEMPHIGUS www.indiandentalacademy.com
  39. 39. MANAGEMENT The aim of treatment in Pemphigus vulgaris is the same as in other autoimmune bullous diseases , which is to decrease the blister formation and promote healing of blisters and erosions and determine the minimal dose of medication necessary to control the disease process. (Ahmed et al.,1980; Becker and Gaspari 1993) www.indiandentalacademy.com
  40. 40. www.indiandentalacademy.com
  41. 41. SYSTEMIC STEROIDS High doses of systemic corticosteroids along with immunosuppressive drugs such as Azithioprine, Dapsone,Cyclosporine initially to clear the lesion Maintain the patient on the low dose to control the condition (Mourellou et al.,1995; Bystryn and Steinman,1996; Korman 2000) www.indiandentalacademy.com
  42. 42. ALTERNATIVE TREATMENT TO CORTICOSTEROIDS DRUGS: 1) Mycophenolate Mophetil 2) Rituximab 3) Etanercept 4) Immunoglobulins 5) Dapsone 6) Cyclosporine THERAPIES: 1) Plasmapheresis 2) Extracorpeal Photochematherapy 3) Immunoadsorption. www.indiandentalacademy.com
  43. 43. MANAGEMENT www.indiandentalacademy.com
  44. 44. CONCLUSION In this case report, we describe the management of a patient who had previously undergone treatment , whose complaints were not relieved for a long time. We also distinguish the diagnosis of oral pemphigus vulgaris and other similar oral lesions and the importance of role of oral physician in early diagnosis and treatment. www.indiandentalacademy.com
  45. 45. BIBLIOGRAPHY 1. Mignogna et al: Oral pemphigus: long term behaviour and clinical response to treatment with deflazacort in sixteen cases. J Oral Pathol Med 1999;29:145-52. 2. Tsuruta D, Kobayashi H: Recent Patents in Pemphigus Research, Prophylaxis,Diagnosis and Treatment in USA (1988-2006). Recent Patents on Inflammation & Allergy Drug Discovery 2007;1:77-81. 3. Azimi H, Golforoushan F: Comparing the Results of Classic Treatment of Pemphigus Vulgaris with that of Cyclophosphamide and Corticosteroid Pulse Therapy. 4. Azizi A, Lawaf S: Oral pemphigus: The management of oral mucous membrane pemphigoid with dapsone and topical corticosteroid. J Oral Pathol Med 2008;37:341-344. 5. Shafer, Hine, Levy. Textbook of Oral pathology. Fifth edition. 2005 Elsevier. 6. Malcolm A, Vernon J B, Martin S G. Burket’s Oral Medicine-Diagnosis & Treatment. Ninth edition. 1994 J. B. Lippincott Company. 7. Neville W B et al.Oral & Maxillofacial Pathology. Second edition. 2002 Saunders. www.indiandentalacademy.com
  46. 46. www.indiandentalacademy.com

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