Vesicular Bullae and Desquamation Questions

Question 1

What is bullous pemphigoid?

Answer 1

A relatively uncommon chronic, autoimmune/inflammatory, subepidermal (under epidermis), and blistering disease (usually in unhealthy elderly patients with comorbidities)

Can last for months/years

Question 2

What is the pathophysiology of bullous pemphigoid?

Answer 2

Antibodies attach to the basement membranes of skin/activate inflammation

Question 3

What are the clinical features of bullous pemphigoid?

Answer 3

Tense bullae on any part of the skin- usually flexor surfaces/folds
Acute/subacute onset (< or = 6 weeks)
Intense pruritis (itching) - broken skin (more chance of infection)
May occur in the mouth (not common)
No scarring
May be a prodrome (may indicate onset of future urticaria, papule, or eczema erruption)

Question 4

How is bullous pemphigoid diagnosed?

Answer 4

By diagnostic studies like:
**Skin biopsy (2 REQUIRED)
biopsy from blister (direct histology): highlights antibodies deposited in blister
direct immunofluorescence (NORMAL SKIN): highlights antibodies in normal appearing skin around blister

blood testing if unable to biopsy- (indirect immunofluorescence to look for antibodies in circulation) *not as reliable

Question 5

What does the treatment of bullous pemphigoid depend on?

Answer 5

The extent of the disease, hard to tell if their disease will progress so usually always put on oral steroids (first line)

Question 6

What are the first line treatments for bullous pemphigoid?

Answer 6

Oral steroids like prednisone (works faster) OR oral antibiotics (tetracyclines, work well for weaning off of steroids, safer for longer term therapy)
»>decreases inflammation cause by attachment of antibodies

Question 7

What is the second line treatment for bullous pemphigoid?

Answer 7

Topical steroids are given along with systemic (oral) meds for relief of itching if needed - only used locally on extremely itchy blisters to prevent overuse of steroids (coupled with oral=can get a ton of steroids in system)

Immunosuppressants (methotrexate, dapsone, imuran) if disease has progressed enough and non-responsive to other therapy: need to make sure you’re trying to prevent infection

REMEMBER: IMMUNOSUPPRESSANT= HIGHER RISK OF INFECTION

Question 8

What is pemphigus (vulgaris)?

Answer 8

Also an autoimmune blistering condition yet it effects mucous membranes more than bullous pemphigoid, in younger patients with malignancy

Question 9

What’s the difference between bullous pemphigoid and pemphigus?

Answer 9

Pemphigus blisters are:
-more painful than itchy, not as large, fragile/less tense
-more common in younger patients/associated with malignancy
-associated more with mucous membranes

Question 10

What is erythema multiforme?

Answer 10

Two types: minor and major
Minor: skin involvement/mild mucous membrane involvement
-may be response to flare of HSV (herpes simplex virus)
Creates symmetric, erythematous target-like skin lesions

Question 11

What is the characteristic lesion of erythema multiforme?

Answer 11

Target lesion: erythematous ring with clearance of center
Scaly, can be itchy
Different than tinea: in erythema multiforme will be a ring within a ring (tinea=only one ring)

Question 12

Where is erythema multiforme usually found?

Answer 12

can occur anywhere
affinity for extensor surfaces: palms, soles
mucous membranes (minor involvement like mouth/lips)
Systemic: if on right, likely will also be on left

Question 13

How is erythema multiforme diagnosed?

Answer 13

Can be biopsied, but usually diagnosed by target lesions
(different than lyme disease: scaly, and multiple rings with erythema multiforme/ lyme disease = one target lesion, not scaly, will have different relating symptoms not seen in erythema)

Question 14

What are the treatments available for erythema multiforme?

Answer 14

Supportive treatment
Topical steroids for specific lesions
If mucous membrane/mouth involvement: viscous lidocaine
Make sure to educate on fluids to prevent dehydration

May need to address suppression of HSV: recurrent flares may require suppressive dosing (antivirals- not a treatment for E.M. itself)

Question 15

What is Stevens Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN)?

Answer 15

(Erythema multiforme major)
An uncommon immune system response that creates blisters in skin and mucous membranes of any system, epidermal detachment, and epidermal necrosis
SJS <10% body surface area
TEN >30% body surface area

Question 16

What causes SJS/TEN?

Answer 16

Medications are a common cause (generally new meds)

Others include: HIV infection, malignancy, idiopathic or unknown cause

Question 17

What are common symptoms of SJS TEN

Answer 17

Fever, orthostasis, tachycahrdia, hypotension, altered level of consciousness, epistaxis, conjuctivitis, corneal ulcerations, erosive vulvovagintis/balantitis, seizures/coma

Question 18

How do you diagnose SJS/TEN?

Answer 18

Biopsy should be done,
but if suspecting of the disease, need to act quickly
-CBC panel
-metabolic panel
-cultures
***these are used to check for infection and function of organs (chance of sepsis)

Question 19

What are the clinical features of SJS/TEN?

Answer 19

Flu like symptom prodrome (onset): productive cough, headache, malaise, fever, arthralgias
**can be misdiagnosed as a viral illness before skin lesions present

Mucous membranes will be affected before skin
Occular, oral, GI, Pulm, Genitourinary systems (pain in these areas)

Starts as flat spots that evolve into blisters which necrose (peeling of the skin, concern for secondary infection): painful, nut usually itchy

Question 20

How is SJS/TEN managed/treated?

Answer 20

-In an ICU/burn unit if possible
-Discontinue any medication that is causing the disease (generally new med)
-giving fluids/electrolytes
-keep skin moist/wet dressings
-consults from other specialties (if other systemic involvement)
-intubation in respiratory compromise
-pain control
-address any secondary infection caused by disease

Question 21

What medications are commonly associated with SJS/TEN?

Answer 21

Antibiotics (sulfa), NSAIDS, anti-gout medications, psychoepileptics

Question 22

What is the difference between SJS/TEN?

Answer 22

The amount of skin affected
1-10% for SJS
10-30% for TEN

Question 23

What are possible long term complications of SJS/TEN?

Answer 23

Occular: Photophobia (sensitivity to light)/Blurred vision
Skin: scarring/Pigment changes (melanocytes: hypo- or hyper-pigmentation when healed)
Nails: loss of nails
GI: Esophageal strictures-narrowing from inflammation and scarring (hard time eating/drinking)
Oral/genital: chronic erosions

Question 24

What is the SCORTEN score for SJS/TEN?

Answer 24

A scoring scale, the higher the score the worse the prognosis
Done during initial presentation and 3 days after initial admission

(points for things like high surface area, old age, comorbidities, septic symptoms)

Question 25

What is the positive Nilosky sign?

Answer 25

Edges of a blister which look like intact/normal skin
will easily slough off with light pressure

Question 26

What is the prognosis/mortality of SJS/TEN determined by?

Answer 26

Type of disease is determined by extent of body surface area (skin sloughing)
1-10% for SJS
30-50% for TEN

The higher the body surface area affected, the higher chance of severity/mortality

Age, existing diseases (comorbidities) will play a role here. If death occurs, usually because of sepsis/multiorgan failure.

Question 27

How do you calculate body surface area using the rule of 9’s?

Answer 27

Sections/groups of total body surface area in percentages that are divisible by 9

Back/front of head and neck: one side: 4.5%, together: 9%

front/back of one arm &hand: one side: 4.5%, together: 9%, both arms: 18%

front/back of one leg &foot: one side: 9%, both sides: 18%, both legs: 36%

genitals: 1%

abdomen/lower back: one side: 9%, both sides: 18%

chest/upper back: one side 9%, both sides: 18%

**IF YOU FORGET: REMEMBER PALM IS 1% OF TOAL BSA