chronic blistering disease part i rick lin, do mph second year resident
Post on 20-Dec-2015
220 views
TRANSCRIPT
![Page 1: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/1.jpg)
Chronic Blistering Chronic Blistering Disease Part IDisease Part I
Rick Lin, DO MPH
SECOND year resident
![Page 2: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/2.jpg)
![Page 3: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/3.jpg)
![Page 4: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/4.jpg)
![Page 5: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/5.jpg)
![Page 6: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/6.jpg)
![Page 7: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/7.jpg)
![Page 8: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/8.jpg)
![Page 9: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/9.jpg)
![Page 10: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/10.jpg)
![Page 11: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/11.jpg)
![Page 12: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/12.jpg)
Pemphigus Vulgaris
Easily rupture bullae Bulla is clear at first but may become
hemorrhagic or even seropurulent then form erosion
Appear first in the mouth and them commonly in the groin, scalp, face, neck, axillae, or genitals
Nikolsky sign is present
![Page 13: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/13.jpg)
Nikolsky sign vs. Asboe-Hansen
Nikolsky sign: absence of cohesion in the epidermis, upper layers are easily made to slip laterally by slight pression or rubbing.
Asboe-Hansen sign: direct pression on intact bulla leading to bulla-spread phenomenon
![Page 14: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/14.jpg)
Pemphigus Vulgaris
Mouth lesion first appear in 60% of the case
Mucosa with painful erosion Mouth odor is offensive and penetratingly
unpleasant Esophagus may be involved and sloughing
of entire lining to form a cast (esophagitis dissecans superficialis)
![Page 15: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/15.jpg)
Pemphigus Vulgaris
Epidemiology: M=F, usually 5th and 6th decades, rare in young person
Etiology: autoimmune blistering disease mediated by intercellular antibodies
IgG throughout epidermis, C3 reliably found
Desmoglein-3 antibody detected
![Page 16: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/16.jpg)
Pemphigus Vulgaris
Drugs which induces pemphigus– Penicillamine for treatment of RA, most often
for foliaceous type– Captopril, penicillin, thiopronine, interleukin-
2, rifampin
![Page 17: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/17.jpg)
![Page 18: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/18.jpg)
![Page 19: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/19.jpg)
![Page 20: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/20.jpg)
![Page 21: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/21.jpg)
The hallmark of pemphigus is the finding of IgG autoantibodies directed against the cell surface of keratinocytes. A Pemphigus vulgaris sera containing anti-desmoglein 3 IgG alone stain the cell surfaces in the lower epidermis. B Pemphigus vulgaris sera containing both anti-desmoglein 3 IgG and anti-desmoglein 1 IgG stain the cell surfaces throughout the epidermis. C Pemphigus foliaceus sera, which contain only anti-desmoglein 1 IgG, stain the cell surfaces throughout the epidermis, but more intensely in the
superficial layers.
![Page 22: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/22.jpg)
![Page 23: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/23.jpg)
![Page 24: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/24.jpg)
Pemphigus Vulgaris
Treatment:– Topical: Silvadene, Maalox for mouth– Systemic: Prednisone 60mg to 100mg daily– Systemic: Azathioprine (Imuran), cyclophasphamide
(Cytoxin), Mexotrexate maybe used in combination– Repeat pemphigus antibody titer in 4-8 weeks after
treatment. If not improving, increase prednisone up to 150mg/day
– Solu-medrol IV pulse therapy at 1g/day over 2-3 hour period, repeat for 5 days if patient is not responding orally.
![Page 25: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/25.jpg)
Pemphigus Vulgaris
Use of an immunosuppresant is helpful in diminishing the need for corticosteroids.
Imuran is one of the best Risk of death in pemphigus from side
effect of oral prednisone is greater than the risk of death from the disease itself
![Page 26: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/26.jpg)
![Page 27: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/27.jpg)
Pemphigus Vegetans
A variant of pemphigus vulgaris Characterized by flaccid bullae that become
erosions and form fungoid vegetation or papillomatous proliferations, especially in body folds
Histology finding are identicla with those of pemphigus vulgaris, but there is an increased papillary proliferations and marked epidermal hyperplasia.
![Page 28: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/28.jpg)
![Page 29: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/29.jpg)
![Page 30: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/30.jpg)
![Page 31: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/31.jpg)
Pemphigus Foliaceus
Mild, chronic variety of pemphigus characterized by flaccid bullae and generalized or localized exfoliation.
Nikolsky sign present. Oral lesions rarely seen.
Desmoglein-1 antibody. Patient with Pemphigus Foliaceus are not
severely ill.
![Page 32: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/32.jpg)
![Page 33: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/33.jpg)
![Page 34: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/34.jpg)
![Page 35: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/35.jpg)
![Page 36: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/36.jpg)
Fogo Selvagem
AKA Brazilian Pemphigus Endemic form of pemphigus foliaceus
found in tropical regions Histologically and immunohistologically
identical to pemphigus foliaceus
![Page 37: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/37.jpg)
Pemphigus Erythematosus
Senear-Usher Syndrome Resemble lupus erythematosus Positive for lupus band in 80% of patients Histologically resemble Pemphigus
foliaceus Dosage of prednisone required for control
usually is much lower than that of Pemphigus Foliaceus
![Page 38: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/38.jpg)
![Page 39: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/39.jpg)
Paraneoplastic Pemphigus
Mucosal lesions may present as lichenoid with Stevens-Johnson-like presentation
Skin lesions may appear as erythematous macules, lichenoid lesions, erythema multiforme-like lesion, flaccid bullae, and erosion.
Immunohistopathologic reveals IgG and C3.
![Page 40: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/40.jpg)
The characteristic clinical feature is severe intractable stomatitis that extends onto the vermilion lip.
![Page 41: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/41.jpg)
![Page 42: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/42.jpg)
Intraepidermal Neutrophilic IgA Dermatosis
Generalized flaccid bullae, which may rapidly ruptured and crusted
Histological finding shows neutrophilic exocytosis and in some areas neutrophils arranged in a linear fashion at the dermal-epidermal junction.
Direct IF showed an intrercellular deposition of IgA with in epidermis with minimal staining of basal layer.
![Page 43: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/43.jpg)
Intraepidermal Neutrophilic IgA Dermatosis
Second subset of patients develop disease that more closely simulates subcorneal pustular dermatosis
Present much more like Sneddon-Wilkinson patient with serpiginous and annular pustules
Treatment with Dapsone is often effective at as low as 25mg per day.
![Page 44: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/44.jpg)
![Page 45: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/45.jpg)
![Page 46: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/46.jpg)
Bullous Pemphigoid
Large bullae When rupture, shows large denuded area and do
not materially increase in size. Denuded areas show a tendency to heal
spontaneously Begins at a localized site, frequently the shin Young girls maybe initially seen with localized
vulvar erosion and ulcers that resemble signs of child abuse.
![Page 47: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/47.jpg)
Bullous Pemphigoid
Occurs most frequently in the elderly. Age of average onset is 65 to 75 years Etiology: circulating basement membrane zone
antibodies of the IgG class present 70%. Site of IgG binding has been localized to the
lamina lucida, with accentuation near hemidesmosome
Bullous pemphigoid antigen 1 (BPAg1) and 2 (BPAg2) identified in 90% of patients
![Page 48: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/48.jpg)
![Page 49: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/49.jpg)
![Page 50: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/50.jpg)
![Page 51: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/51.jpg)
![Page 52: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/52.jpg)
![Page 53: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/53.jpg)
![Page 54: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/54.jpg)
![Page 55: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/55.jpg)
![Page 56: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/56.jpg)
![Page 57: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/57.jpg)
![Page 58: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/58.jpg)
![Page 59: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/59.jpg)
![Page 60: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/60.jpg)
![Page 61: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/61.jpg)
![Page 62: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/62.jpg)
![Page 63: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/63.jpg)
![Page 64: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/64.jpg)
A Direct immunofluorescence microscopy studies of perilesional skin demonstrating linear continuous deposits of IgG along the epidermal basement membrane zone (arrow). The same pattern of labeling is observed in cicatricial pemphigoid and epidermolysis bullosa acquisita. B Indirect immunofluorescence microscopy study utilizing salt-split normal human skin as a substrate. Patient's IgG autoantibodies are bound to the epidermal side (roof) of the split (arrow). The level of the artificial separation is indicated by asterisk. Cell nuclei are stained blue. The same pattern of labeling is observed in a subset of patients with cicatricial pemphigoid .
![Page 65: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/65.jpg)
Treatment
Same treatment for pemphigus, with the expectation that disease will respond readily with lower dose of corticosteroid.
In severe case, pulse therapy with methylprednisolone giving 15mg/kg in 16 ml bacteriostatic water over period of 30 to 60 minutes daily for 3 doses.
Imuran is commonly used in resistant cases
![Page 66: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/66.jpg)
Treatment
Additional treatment options include mexotrexate and mycophenolate mofetil.
Nicotinamide 500mg three times daily combined with tetracycline 500mg four times daily works 10 out of 14 patients.
![Page 67: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/67.jpg)
![Page 68: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/68.jpg)
![Page 69: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/69.jpg)
![Page 70: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/70.jpg)
![Page 71: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/71.jpg)
![Page 72: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/72.jpg)
![Page 73: Chronic Blistering Disease Part I Rick Lin, DO MPH SECOND year resident](https://reader036.vdocuments.us/reader036/viewer/2022062320/56649d4a5503460f94a27282/html5/thumbnails/73.jpg)