CASE PRESENTATION Bullous Pemphigoid .pptx

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1. CASE PRESENTATION Presenter: Dr Anirudh P Moderator: Dr Asha G S Dr Revathi T N Date: 30/12/20

2. Preliminary data: Name : Muniyappa Age: 83 years Sex: Male Address: Anchepalya , Bengaluru Education: no formal education Occupation: formerly carpenter

3. Chief complaints: Itchy lesions with blistering over the upper limbs x 1 month Multiple blisters trunk, extremities, neck and genitalia x 10 days

4. History of presenting illness: Patient was apparently alright 1 month back, when he started developing reddish itchy lesions with blistering over the forearms insidiously. Lesions were increasing in number day to day Itching was severe in nature, disturbing his routine activities and sleep. It was continuous with no apparent triggering factors or diurnal variation. There were no aggravating or relieving factors. He developed wounds secondary to scratching. He was brought to dermatology opd at Victoria hospital. He was evaluated and treated with topical medications. He was advised to report if lesions worsened. After 10 days of the visit, he started developing blisters on both legs, thighs, forearms, neck. Blisters were increasing in number over the period.

5. They were tense in nature, contained clear fluid and did not rupture spontaneously. They lasted for few days and ruptured to leave behind raw areas. No h/o fever , joint pain. No h/ o o ver the counter topical remedies, drugs No h/o trauma, surgery, burns No h/o weight loss No h/o photosensitivity

6. Past history: No h/o similar complaints in the past K/c/o T2 DM since 20 years on Metformin 500 mg BD, Glimeperide 2 mg BD Not a k/c/o HTN, asthma, tuberculosis, epilepsy and other neurological diseases,

7. Treatment history: H/o treatment under dermatologist at Victoria hospital prior to flare up of the disease. Skin biopsy was performed from the lesional skin for histopathology and perilesional skin for Direct Immunofluorescence (DIF). Biopsy was suggestive of Bullous pemphigoid and DIF was corroborative of Bullous pemphigoid. He was treated with topical clobetasol propionate 0.05% cream and advised to report if lesions aggravated.

8. Personal History: Diet: Mixed Appetite: Reduced Sleep: disturbed Bowel and bladder habits: normal Addictions: None

9. Family history: Married and has three children No h/o blistering diseases in family All the family members are healthy

10. General physical examination: Patient is an elderly male, who is moderately built and moderately nourished. He is oriented to time, place and person. Pallor: absent Icterus: absent Cyanosis: absent Clubbing: absent Lymphadenopathy: absent Edema : absent

11. Systemic examination: Respiratory system: Bilateral normal vesicular breath sounds heard, No added sounds Cardiovascular system: S1, S2 heard, No murmurs Per abdomen: Soft, non tender, bowel sounds heard Central nervous system : higher mental functions present

12. Cutaneous examination: Scalp: tonsured with greying of hair Face: Multiple discrete tense bullous and vesicular lesions seen over the right temple, forehead, periorbital regions, pinnae. Multiple crusted erosions, both discrete and coalesced were present over the forehead, bilateral malar regions, left mandibular region, bilateral pinnae, retro-auricular regions.

13. Neck: Multiple erosions seen coalesced over nape of neck, anterior part of neck with crusting over few places Chest: Multiple erosions see coalesced over bilateral supramammary region, sternal region Abdomen: Multiple coalesced erosions with crusting seen over umbilical and bilateral lumbar regions Back: Multiple erosions seen coalesced over upper back

14. Upper limbs : Multiple erosions both discrete to coalesced seen over bilateral dorsum of hands, forearms, arms, axillary, infra-axillary regions. Palms are uninvolved. Lower limbs: Multiple erosions with interspersed vesicles and bullous lesions seen involving bilateral thighs, upper one third of legs. Dorsum of feet and soles were uninvolved. Few erosions were seen in bilateral groin region

15. Genitalia: few erosions were present over scrotum Mucosa: Oral: crusted erosions were seen over both upper and lower lips, rest of the oral mucosa was uninvolved Nasal, conjunctival, genital: appeared to be normal Nails: appeared to be normal Nikolsky sign : Negative Bulla spread sign : positive with uniform rounded edges

16. Clinical diagnosis: ? Bullous pemphigoid ? Linear Ig A Bullous Disease ? Inflammatory Epidermolysis Bullosa Aquisita

17. Management: Patient was advised admission and in patient care Investigations: CBC, AEC,PBS,ESR, urine routine, RFT, LFT, serum electrolytes, Viral serology-HIV, HBsAg, HCV, CXR Tzanck smear, Skin biopsy for HPE, and DIF Results: Moderate anemia Leucocytosis with eosinophilia Raised ESR RFT was within normal limits LFT- hypoalbuminemia

18. Negative for all viral serologies CXR: within normal limits Tzanck smear: mixed inflammatory infiltrate with no acantholytic cells or giant cells HPE: Epidermis: subepidermal clefting with mixed inflammatory infiltrate consisting of eosinophils, neutrophils and lymphocytes. No acantholytic cells were present. Dermis: superficial perivascular lymphocytic infiltrate seen DIF: Deposition of IgG and C3 along dermo-epidermal junction Final diagnosis: Bullous pemphigoid with T2DM

19. Physician opinion was taken in view of starting dexamethasone-cyclophosphamide pulse therapy Necessary investigations- ECG, echocardiography were performed. They were within normal limits

20. Treatment: IV dexamethasone 8mg OD for 1 week followed by one cycle of dexamethasone-cyclophosphamide-pulse therapy. 100 mg of Inj. dexamethasone in 500 ml of 5% dextrose with 8 units of regular insulin on Day 1 and Day 3. 500 mg Inj. Cyclophosphamide added on day 2. IV Ceftriaxone 1g BD ATD x 14 days Tablet Tetracycline 500 mg TID for 2 weeks Normal saline compresses for crusted lesions and fusidic acid with beclamethasone cream for erosions Protein supplements Insulin injection and oral hypoglycemic agents were continued

21. Course in hospital and outcome: Uneventful and patient responded to the treatment Discharge advise: Tablet Prednisolone 30 mg for 2 weeks followed by 20 mg for 2 weeks Tablet cyclophosphamide 50 mg OD Capsule omeprazole 20 mg OD Tablet calcium 500 mg OD Tablet ferrous sulphate OD Capsule Multivitamin OD along with protein supplements Patient was advised to follow up after 28 days but failed to follow up due to corona virus pandemic

22. THANK YOU