Chapter 56 - Epidermolysis Bullosa Acquisita

Question 1

TABLE 56-3
Treatments for Epidermolysis Bullosa
Acquisita (EBA)

DOSE RANGE

Colchicine

Answer 1

0.6-3.0 mg/d

Must start with 0.4 to 0.6 mg/d and each 1 to 2 weeks double this dose as tolerated. When patient develops diarrhea, back off 1 tablet (0.4-0.6 mg).

Question 2

TABLE 56-3
Treatments for Epidermolysis Bullosa
Acquisita (EBA)

DOSE RANGE

Cyclosporine A

Answer 2

6 mg/kg/d

Question 3

TABLE 56-3
Treatments for Epidermolysis Bullosa
Acquisita (EBA)

DOSE RANGE

Dapsone

Answer 3

100-300 mg/d

Begin at 25 mg/d and double each week after the complete blood count and liver function tests. Most patients need between 100 and 250 mg/d. Increasing the dose slowly helps the patient tolerate the anemia that develops (ie, less orthostatic light headedness, etc.). Expect a 1- to 2-g drop in the patient’s hemoglobin on therapeutic doses.

Question 4

TABLE 56-3
Treatments for Epidermolysis Bullosa
Acquisita (EBA)

DOSE RANGE

Cytoxan

Answer 4

50-200 mg/d

Question 5

TABLE 56-3
Treatments for Epidermolysis Bullosa
Acquisita (EBA)

DOSE RANGE

Prednisone

Answer 5

1.0-1.5 mg/kg

Usually does not help the classic, mechanobullous type of EBA with minimal inflammation. However, it may be somewhat helpful in the bullous pemphigoid–like type of EBA.

Question 6

TABLE 56-3
Treatments for Epidermolysis Bullosa
Acquisita (EBA)

DOSE RANGE

Intravenous immunoglobulin

Answer 6

3 g/kg divided over 5 d

Intravenous immunoglobulin is given over 4 to 5 days every month for 5 or 6 months to give it an adequate trial.

Question 7

TABLE 56-3
Treatments for Epidermolysis Bullosa
Acquisita (EBA)

DOSE RANGE

Infliximab

Answer 7

5 mg/kg at 0, 2, 4, and 6 wk

Question 8

TABLE 56-3
Treatments for Epidermolysis Bullosa
Acquisita (EBA)

DOSE RANGE

Rituximab

Answer 8

375 mg/m2 of BSA, IV weekly × 4 wk
Or 1000 mg IV on week 1 and week 3

Question 9

TABLE 56-3
Treatments for Epidermolysis Bullosa
Acquisita (EBA)

MEDICATION

When patient develops diarrhea, back off 1 tablet (0.4-0.6 mg).

Answer 9

Colchicine

Question 10

TABLE 56-3
Treatments for Epidermolysis Bullosa
Acquisita (EBA)

MEDICATION

Increasing the dose slowly helps the patient tolerate the anemia that develops (ie, less orthostatic light headedness, etc.).

Answer 10

Dapsone

Question 11

TABLE 56-3
Treatments for Epidermolysis Bullosa
Acquisita (EBA)

MEDICATION

Usually does not help the classic, mechanobullous type of EBA with minimal inflammation. However, it may be somewhat helpful in the bullous pemphigoid–like type of EBA.

Answer 11

Prednisone

Question 12

TABLE 56-3
Treatments for Epidermolysis Bullosa
Acquisita (EBA)

MEDICATION

Given over 4 to 5 days every month for 5 or 6 months to give it an adequate trial

Answer 12

Intravenous immunoglobulin