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An 82-year-old woman comes to your office with a complaint of blistering skin rash. She reports that the blisters started to develop approximately 3 months ago, and at that time a friend told her that it looked like œhives. The blistering process has continued, however, and she now reports getting 2 or 3 new blisters every day. The blisters are widespread and most numerous on the flexural surfaces of the skin. They have not, however, appeared on the head, neck, and in the mouth. You order laboratory studies and perform a punch biopsy of a skin lesion. The results of these tests are as follows:
HEAMATOLOGIC:
hg....11.2
hct....34
plt ct...220,000
wbc....9850
BLOOD/SERUM CHEMISTRIES
alkaline phosphatase....101
alt......79
ast.....83
creatinine....102
k.....4.2
na....138
bun.......19
SKIN BIOPSY: direct immunoflorescence deposits of igG antibodies. and complements C3 in a linear band at the dermo epidermal jxn.
Which of the following is the most appropriate therapy for this patient?
A. Methotrexate
B. Oral prednisone
C. Oral prednisone and plasmapheresis
D. Tetracycline
E. Topical steroids
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The correct answer is E. This patient has bullous pemphigoid and topical steroids are the treatment of choice. Bullous pemphigoid is a relatively uncommon autoimmune disease of the skin in which IgG antibodies are directed at the dermal-epidermal junction. The disease tends to affect older individuals, who present much as the patient in this question: with tense blisters most prominent on flexural surfaces and sparing of the head, neck, and mouth. Peripheral blood eosinophilia sometimes is seen, and skin biopsy, as in this case, reveals IgG antibodies deposited at the dermal-epidermal junction. Although systemic steroids were long considered to be the treatment of bullous pemphigoid, research has shown that high-potency topical steroids (e.g., clobetasol) applied to the skin in large quantities results in better outcomes with fewer complications than do systemic steroids, even for severe disease. Indeed, in comparison with systemic steroids, topical steroids result in better control of disease with higher survival rates than treatment with systemic steroids. If a patient with bullous pemphigoid is able to comply with a topical steroid regimen, topical steroids are thus the treatment of choice.
Methotrexate (choice A) has been used for bullous pemphigoid, but it is not a first-line therapy.
Oral prednisone (choice B), as noted, in the past was standard therapy for bullous pemphigoid. Although oral prednisone does provide benefit, complications of systemic steroids are, unfortunately, also responsible for a substantial proportion of the morbidity and death associated with bullous pemphigoid. Research has demonstrated that better control of bullous pemphigoid is achieved with topical steroids rather than oral prednisone, and the complication rates are lower with topical steroids.
Combined oral prednisone and plasmapheresis (choice C) have been tried for treatment of bullous pemphigoid. Research has shown that plasmapheresis does not add additional benefit to that derived from prednisone.
Tetracycline (choice D) has been used for treatment of bullous pemphigoid. It is not, however, a particularly effective therapy for extensive or widespread disease, nor is it a first-line therapy.
