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Bullous Pemphigoid
1) The patient, a 45-year-old female, presented with multiple fluid-filled itchy lesions over her limbs and face for the past week and developed oral lesions 3 days after admission. 2) A clinical diagnosis of bullous pemphigoid was made based on tense fluid-filled cutaneous lesions, involvement of oral mucosa, histopathology showing subepidermal blistering, and direct immunofluorescence demonstrating linear deposition of IgG and C3 along the basement membrane zone. 3) The patient was treated with systemic corticosteroids and immunosuppressants along with topical corticosteroids for oral lesions and advised long-term follow-up to manage the chronic autoimmune condition.
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Bullous Pemphigoid
- 1. ORAL VESICULO-BULLOUS LESION :A case presentation
- 2. INTRODUCTION Auto-immune, mucocutaneous conditionscan frequently present with oral lesions. The severity of these lesions can have a dramatic impact on the quality of life of a patient. The clinical presentation of these lesions may vary from minute vesicles or bullae to multiple erosive or ulcerative areas. Hence, it is important to know the distinguishing features of such lesions to reach a conclusive diagnosis.
- 3. A 45 yearold female patient named Samyukhta, complained of multiple fluid- filled itchy lesions for the past 1 week over her limbs and face. CHIEF COMPLAINT
- 4. Severe itchy lesions overthe limbs 4 months back. Progressed to form fluid-filled lesions. Patient took treatment in a nearby hospital No improvement in the lesions Resorted to ayurvedic treatment Complete remission of the lesions. Since past 10days, exacerbation in the lesions Noticed lesions over her limbs, face and inside her mouth. HISTORY OF PRESENT ILLNESS
- 5. ? Previous episode oflesions ? History of lesions in the eye ? History of scarring in the affected areas ? History of trauma in the sites affected before lesions appeared HISTORY OF PRESENT ILLNESS
- 6. History of takinganti- cholesterol drugs since past 6 months. History of severe weight loss in the past 1 year (10-12 kg in 1 yr) PAST MEDICALHISTORY FAMILY HISTORY PAST DENTALHISTORY Not significant Not significant
- 7. PERSONALHISTORY Vegetarian diet, goodappetite Sleep: undisturbed Brushes twice daily with a brush and toothpaste. No adverse habits reported.
- 8. EXAMINATION OF THEPATIENT
- 9. GENERALEXAMINATION -Moderate built -Moderately nourished -Alert -Conscious -Cooperative Pallor (+) Icterus (-) Cyanosis(-) Clubbing (-) Lymphadenopathy(+) Edema (-) VITAL SIGNS:- -Pulse: 80 / minute -B.P: 120/80 mm Hg -Respiratory rate:15 breaths/ minute. -Temperature:Afebrile
- 10. • Multiple areasTense, fluid- filled lesions CUTANEOUS EXAMINATION
- 11. Abdomen Relative sparing ofpalms and soles CUTANEOUS EXAMINATION Right submandiibular lymphnodes palpable, non-tender, mobile
- 12. On the dayof admission No oral lesions were noted 3 days later the patient developed: INTRA-ORAL( Soft Tissue) EXAMINATION
- 13.
- 14. Pemphigus vulgaris Lichen Planus Pemphigoides Linear IgA disease Epidermolysis BullosaAcquisita DIFFERENTIALDIAGNOSIS
- 15. Hb- 12 gm/dl TLC-11,600/ mm3 DLC- N- 70.4 % L- 11.2% E- 12.4 % M- 5.5 % PLATELETS - 2.33 l/ mm3 RBS - 78mg/dl UREA - 12mg/dl CREATININE - 0.7mg/dl TOTAL PROTEIN - 5.8g/dl ALBUMIN - 3.1g/dl INVESTIGATIONS & RESULTS
- 16. Na+- 141mEq/L K+- 3mEq/L Cl--102mEq/L HCO3 --23.6mEq/L Ca2+ - 8.4mEq/L PO4 3- - 5.2mEq/L Total bilirubin- 0.3 Direct bilirubin- 0.13 SGOT- 27 IU/L SGPT- 23IU/L ALP- 132IU/L ESR- 23 INVESTIGATIONS & RESULTS
- 17.
- 18. • Linear stainingof Basement membrane zone (BMZ) with IgG & C3 IMMUNOFLUORESCENCE TEST
- 19.
- 20. Medications administered whenpatient was admitted. • Inj AVIL/ Pheniramine (1cc/ ½ amp) i.m. 1-0-1 x 3days • Inj Ceftazidime (1g) i.v. 1-0-1 x 3days • Inj Ranitidine(1amp) i.v. 1-0-1 x 3days • Tab Prednisolone 20mg 1-1-0 x7 days • Cap Omeprazole 20mg 1-0-1 • Tab Callum 500mg 0-1-0 • Tab Amlodipine 5mg 1-0-0 x 3days TREATMENT
- 21. • Treatment forthe oral lesions was started on the 2nd day of appearance of oral lesions. • Topical 0.1% Triamcinolone acetonide ointment 1-1-1 x 7days • Mouthwash Lidocam 1-1-1 x 7days TREATMENT
- 22. • Patient wasadvised to take the following medication after discharge • Tab Wysolone 20mg 1-0-0 x 5days • Tab Wysolone 10mg 0-1-0 x 5days • Cap Omez 20mg 1-0-1 x 7days • Tab Callum 500mg 0-1-0 • Tab Amlong 5mg 1-0-0 TREATMENT
- 23. • INTRODUCTION- • BullousPemphigoid (BP) is a blistering disease. • Initially • Later large, tense blisters develop on both • NORMAL & • ERYTHEMATOUS areas. • Mucosal involvement is rare presents with blisters & erosions Pruritis Urticaria Erythema DISCUSSION
- 24. • PATHOGENESIS- • Autoantibodieshemidesmosomes-associated proteins. • BP230, BP180 2 major antigens associated with bullous pemphigoid. Auto-antibody deposition leads to blistering Disruption of HEMIDESMOSOMES “BLISTER FORMATION” DISCUSSION
- 25. • CLINICAL FEATURES- •AGE- > 60 years; < 5years ( can occur in < 40years patients) • RISK of developing BP- Increases with Age • ♂ > ♀ • Initially ITCHING, NON- SPECIFIC RASH URTICARIA - LIKE Lasts for 1-3weeks before BLISTERS develop ECZEMATO US NO BLISTERS for several months DISCUSSION
- 26. • CLINICAL FEATURES- •FLUID- • Clear, serous exudate may be blood-stained. • If BLISTERS REMAIN INTACT for several days Jelly-like coagulated fibrin. Erythema may persist at the site of blister for many weeks or months. DISCUSSION
- 27. • ORAL MANIFESTATIONS- •Oral lesions Rare • If they do occur : APPEARANCE- DISCUSSION Small blisters: may remain intact When ruptures ulcerations
- 28. SITE- • Gingival involvement •Exceedingly painful • Erythematous & desquamate minor frictional trauma • Buccal mucosa, palate, floor of the mouth, tongue. DISCUSSION • ORAL MANIFESTATIONS-
- 29. HISTOPATHOLOGICALAPPEARANCE • Bullae- subepidermal •No acantholysis • Epithelium normal • Fibrinous exudate, inflammatory cells ELECTRON MICROSCOPY REVEALS • Basement membrane attached to connective tissue. • Acute phase Leukocytosis & Eosinophilia DISCUSSION
- 30. Clinical Features → Diseases↓ Age (yrs) Cutaneous Involvement Mucosal Involvement Lesions ClinicalSigns Prognosis Bullous Pemphigoi d >60 < 5 Trunk, limbs, flexures Minor Urticated plaques, tense blisters, milia 3-6yrs Pemphigus Vulgaris 50-60 Scalp, face, flexures, may be generalised Always oropharynx, conjunctiva, genital Flaccid blisters, erosions, flexural vegetations Variable, may remit Pemphigus vegetans 40-50 Flexural areas Oral Vesicles, pustules, erosions, vegetating plaques Variable, may remit Cicatricial Pemphigoi d 40-50 Infrequent (30%) Major, severe Erosions, blisters, scarring, gingivitis Chronic DIFFERENTIALDIAGNOSIS
- 31. Clinical Features→ Diseases↓ Age (yrs) Cutaneous Involvement Mucosal Involvement Lesions Clinical Signs Prognosis (yrs) Lichen Planus >60 or < 5 Trunk, limbs, flexures MinorPlaques, 3-6 Chronic Bullous Disease of Childhood Children Face, Trunks, limbs Majority (few severe) Urticated plaques, annular lesions, tense blisters 3-4 Adult Linear Ig A disease Young adults to elderly Trunk, limbs Majority (few severe) Urticated plaques, tense blisters, papulo- vesicles 3-6 Epidermol ysis Bullosa Adults & children Generalised , variable Some (few severe) Urticated plaques, tense blisters, Persists DIFFERENTIALDIAGNOSIS
- 32. 1.) Untreated BullousPemphigoid • Self-limiting disease • Over a number of months or years 2.) Acute Blistering Phase of Bullous Pemphigoid • FATAL 3.)Poor Prognostic factors • Age • Generalised disease • Low Albumin PROGNOSIS
- 33. • Initial Dosage20- 70mg/day • After a few weeks 15-20mg/day • Majority managed <10mg/day TOPICAL & SYSTEMIC CORTICOSTEROIDS • Azathioprine • Methotrexate ( if psoriasis is also present) IMMUNOSUPPRESS ANTS TREATMENT
- 34. Since most ofvesiculo-bullous lesions initially present with oral lesions, we as oral physicians are most often the first ones to notice these lesions Hence, it is important to know the different conditions that can cause these multiple lesions so that an early intervention can be highly beneficial in these debilitating conditions. CONCLUSION
- 36. CASE REPORT SYSTEMIC EXAMINATION •Respiratory System- Normal Vesicular Breath sounds were present • CVS- Normal heart sounds present • P/A- Soft, non-tender • CNS- No abnormality was detected