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Old 06-11-2011
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Skin Flaccid bullae with underlying normal skin ...

A 45-year-old man experiences the gradual eruption of skin blisters over the scalp, face, chest, axillae, and groin. He has a 6-week history of painful oral erosions. He has experienced fatigue and malaise during this time. His previous medical history is unremarkable; he takes no medications and has no known allergies. He is of Mediterranean ancestry, works in an industrial processing plant, drinks 20 to 40 g of ethanol weekly, and does not smoke. There are numerous erosions in the scalp, over the face, axillae, and chest with associated crusts. Additionally, there are several flaccid bullae with underlying normal skin, and painful oral mucosal erosions. Lateral pressure on the skin results in epidermal displacement. What is the most likely course of disease in this patient?

Answer Choices
A. Disease is self-limiting and treatment is not required
B. One year mortality is 75% despite treatment with appropriate therapy
C. The most common cause of death in treated cases is a result of sepsis
D. Treatment with appropriate agents reduces 1-year mortality to 10%
E. Untreated, the 1-year mortality is lower than that for bullous pemphigoid
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Old 06-11-2011
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This is pemphigus vulgaris if i am not wrong.

It is definitely more fatal than bullous pemphigoid (e), and it requires treatment with steroids (a).
So it could be b or c.
I'l go with C
C. The most common cause of death in treated cases is a result of sepsis

As sepsis is the most common cause of death in burns patients, so I am just applying that same concept here.

I just have a problem with 'treated cases'. So it can be B as well.
Lets see what the ans really is!
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Old 06-11-2011
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Default Pemphigus vulgaris-definitely!

Pemphigus vulgaris is an uncommon, potentially fatal, autoimmune disease characterized by intraepidermal blisters and extensive erosions on apparently healthy skin and mucous membranes. Diagnosis is by skin biopsy with direct immunofluorescence testing. Treatment is with corticosteroids and sometimes immunosuppressants.

The sign mentioned in the case is -Lateral pressure on skin adjacent to a blister causes epidermal detachment (Nikolsky's sign).

Before systemic corticosteroids were used, pemphigus vulgaris was usually fatal; most patients died within 5 yr of disease onset.

Severe infection is the most frequent cause of death.


With treatment, the disorder tends to be chronic. Side effects of treatment may be severe or disabling.
Possible Complications

  • Secondary skin infections
  • Severe dehydration
  • Side effects of medications
  • sepsis
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Old 06-13-2011
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Option D (Treatment with appropriate agents reduces 1-year mortality to 10%) is correct. This patient has pemphigus vulgaris. Prior to treatment with steroids and other immune-modulating agents, the 5-year mortality was 100% and the 1-year mortality was 75%. Immunosuppressants have had a major impact and the 1-year mortality is now approximately 10%.

Option A (Disease is self-limiting and treatment is not required) is incorrect. Pemphigus vulgaris is almost uniformly fatal without therapy.

Option B (One year mortality is 75% despite treatment with appropriate therapy) is incorrect. Adequate immunosuppressive therapy has lowered the mortality to near 10%.

Option C (The most common cause of death in treated cases is a result of sepsis) is incorrect. The most common cause of death in treated cases is the result of side effects of therapy. Prior to immunosuppressive therapy, sepsis was the most common cause, as the erosions became secondarily infected.

Option E (Untreated, the 1-year mortality is lower than that for bullous pemphigoid) is incorrect. The 1-year mortality for untreated pemphigus vulgaris is much higher than bullous pemphigoid. With treatment, both have similar 1-year mortality rates.
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Old 06-13-2011
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Default 10% one year mortality in Pemphigus!

It depend on the reference I suppose!

It's good to learn about its poor prognosis and high mortality!

Thanks
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