|Comments from Faculty and Members
David Elpern MD, Williamstown, MA, USA on
Nov 11, 2004
This case demonstrates how important the doctor-patient relationship
is. The patient lives 120 km from you and needs to be seen regularly
and to understand that without your meds he will die. Some of
our older colleagues can remember pemphigus in the era before
steroids. The dermatology wards were full of these patients
just waiting for sepsis.
It will be interesting to hear the comments from some experts
in bullous disease; but also keep in mind that your relationship
with this patient will determine how he does. The treatment
is more straightforward than forming a relationship with this
man. I have confidence that you will forge a truly therapeutic
partnership with him now that he knows what will happen if he
is not compliant. I suspect you will need to see him every couple
of weeks for a few months to make sure he understands the treatment
and trusts you, not the traditional healers in his area.
Khalifa Shaquie MD, PhD, Professor of Dermatology,
College of Medicine, University of Baghdad, Baghdad, Iraq
on Nov 12, 2004
Pemphigus vulgaris is relatively a common social & medical
problem in Iraq, often affecting young adult people. Most patients
do not take their therapy in the right way & some of them
default. All are afraid from the side effects of the drugs.
So public education regarding the disease & therapy is strongly
recommended. All patients should be told that the drug dose
is initally high,then reduced gradually over time & might
be stopped. This might minimise the trauma & make therapy
more easy. I agree about diagnosis & management.
Abir Saraswat MD, Lucknow, India on Nov 12,
This is a very common problem in India and we have faced it,
alas all too often. In this case, the lure of a "permanent"
cure without "toxic" allopathic medicines may have
been too great for the patient to resist. We find that if too
much emphasis is laid on acquainting the patient with adverse
effects of steroids, they get scared, especially in pemphigus,
where large doses are used for long (indefinite) periods.
As regards therapy, I would suggest monthly dexamethasone and
cyclophosphamide pulse therapy (you can substitute methylpred
for dexa), which works beautifully and with minimal side effects.
(Kanwar AJ, Kaur S, Thami GP. Long-term efficacy of dexamethasone-cyclophosphamide
pulse therapy in pemphigus. Dermatology. 2002;204(3):228-31.
Omid Zargari MD, Assistant Professor
of Dermatology, Razi Hospital, Guilan University of Medical
Sciences, Rasht, Iran on Nov 12, 2004
I'm working in an area that pemphigus is not uncommon (we have
about 25 new cases each year). The first line therapy for pemphigus
is systemic steroid, which reduces the responsible autoantibodies,
but with regarding the relatively long half-life of these antibodies
(about 3 weeks), response will occur slowly. Therefore, we should
try to convince the patients about the importance of long-term
In the case of non compliance with long-term hospitalization,
I have two suggestions; 1. Dexamethasone- Cyclophosphamide pulse
therapy. It is effective, but needs closer monitoring. 2. IVIG.
It is safe and usually works good in pemphigus (at least in
early phases), but there is a major drawback: its cost!
Raafah Hayani, MD, Baghdad, Iraq on Nov 13,
We've so many cases of severe pemphigus vulgaris in Iraq since
most of the patients give up treatment after early good response.
We manage these cases in isolated burn unit like room with iv
fluids and under the cover of antibiotics with potassium permanganate
bath 1:8000 with methylprednisolone pulse therapy iv in drip
1Gm daily for three days. All immunosuppressive therapy has
delayed action to spare steroids and I agree with you that imuran
is of little benefit in such cases and sometime we feel that
steroid alone work in such cases. Cyclophosphamide is a good
choice with the pulse therapy
Phung Huynh, MD, Department of Dermatology, NY Medical
College, New York, NY, USA on Nov 13, 2004
Dr Ahmed at Harvard has written multiple studies regarding
IVIG for possible cure of bullous disease refractory to other
treatment or for the desired effect of reducing /stopping prednisolone
therapy. He also discussed his method of treatment on one of
the Dialogues in Dermatology. If you like more specific advice
on this patient or the specifics on how to use IVIG I can get
you in touch with Dr Ahmed's fellow.
Victoria P Werth, M.D., Professor of Dermatology, University
of Pennsylvania School of Medicine, Philadelphia, PA, USA on
Nov 13, 2004
Often when therapy is abruptly stopped for PV, the patient
appears to become more steroid-resistant. Thus treatment is
likely to be a challenge in this case. The dose of steroid seems
appropriate. At times, pulse steroids (1 gm solumedrol/day for
5 days) can very rapidly halt a severe case of unresponsive
PV. The choice of immunosuppressives are outlined well. Cytoxan
does work better, but has more toxicity. If this case is refractory
to improvement with steroids, then cytoxan would be a reasonable
choice as adjunctive immunosuppressive therapy. Sometimes refractory
cases respond with institution of either iVIG (2 gm/montly cycle,
given over five consecutive days each month) or plasmapheresis
3 times a week for several cycles (every few weeks). Plasmapheresis
should be undertaken only after immunosuppression with imuran,
cellcept, or cytoxan has been on board for 3-4 weeks. Otherwise,
there can be a rebound of disease activity after stopping plasmapheresis!
Another potential adjunctive therapy is rituximab, but experience
is limited with this agent in pemphigus vulgaris and it can
be very immunosuppressing and has been associated with atypical
This patient is likely severely depessed, a combination of his
severe disease and the prednisone therapy. This should be addressed
in order to increase the likelihood of compliance to the current
recommended therapies. It is not infrequent for patients to
seek alternative therapies when they are frustrated with their
disease or the side-effects of the therapies they are getting.
The importance of compliance has to be repetitively stressed
to the patient, but clearly there are patients who are unable
to follow through with therapy. These patients do poorly long-term.
Richard Sontheimer M.D., Professor of Dermatology,
University of Iowa Carver College of Medicine, Iowa City, IA,
USA on November 14, 2004
I will play the devil's advocate with respect to diagnostic
possibilities concerning the recrudescence of blistering disease.
Considering the very abrupt and aggressive pattern of blistering
disease reoccurrence and other information given about this
episode, I would first have several questions about the specific
cause of the recurrent episode of blistering disease before
accepting this is a case of recurrent pemphigus vulgaris mucocutaneous
disease activity. Specifically, could there be a superimposed
element of drug-induced (dapsone) hypersensitivity such as bullous
erythema multiforme major (Stevens-Johnson syndrome)? In addition,
could there be an element of staphylococcal scalded skin syndrome
in this immunosuppressed adult?
Had the patient continued taking his prednisone, azathioprine,
and dapsone after discharge from the hospital up until the time
of recurrent skin disease activity? If so, had the doses of
the drugs been significantly lowered immediately prior to re-expression
of blistering disease activity?
The clinical photographs appear to have solitary skin lesions
displaying a targetoid appearance. In addition, the hemorrhagic
crusting of the lips that is present would also suggest a bullous
erythema multiforme reaction although this would also be compatible
with a recurrent episode of pemphigus vulgaris.
The photographs would suggest lip and oral mucosal involvement
during the current episode of recurrent disease. Specifically,
it would be important to know whether the ocular conjunctivae
were also inflamed during the recurrent episode? If so, this
would not be compatible with recurrent pemphigus vulgaris but
would be expected to be a component of an erythema multiforme
major reaction. If this man does have a component of Stevens-Johnson
syndrome, is there any evidence that he might have HIV infection
as a predisposing factor for the development of a Stevens-Johnson
syndrome pattern of hypersensitivity?
Was the skin biopsy showing suprabasalar acantholysis taken
from the original diagnostic skin biopsy or from skin lesions
occurring during the period of recrudescent disease? If a skin
biopsy taken during recrudescent skin disease activity showed
a more superficial form of acantholysis, this would be supportive
of a superimposed staphylococcal scalded skin syndrome. Was
direct immunofluorescence microscopy examination carried out
on the original and/or any follow-up skin biopsies?
Regarding management, if there is any evidence of drug- or infection-
(herpes simplex, Mycoplasma pneumonia) induced erythema multiforme
major, then withdrawal of suspected drugs and treatment of associated
infections should be carried out. In addition to aggressive
parenteral antibiotics covering staphylococcal infection (including
the possibility of methicillin-resistant staphylococcus), high
dose corticosteroids, cyclophosphamide, and anti-CD 20 monoclonal
antibody, consideration might also be given to high dose intravenous
immunoglobulin therapy as this could be of benefit in either
recrudescent pemphigus vulgaris or the Stevens-Johnson syndrome.
Best on luck with this most challenging case.
Sunil Dogra MD, Dept. of Dermatology, Postgraduate
Institute of Medical Education and Research, Chandigarh, India
on Nov 16, 2004
Pemphigus is a very common bullous disorder in India. At any
point there are 4-5 patients admitted at our centre for management.
There seems to be no doubt in diagnosis and clinical photographs
are convincing. Regarding therapy we have very good experience
with Dexamethasone - cyclophosphamide pulse (DCP) therapy as
mentioned by Dr Abir Saraswat. However, septicemia compounded
by DCP in such setting of widespread eroded skin is a potential
practical problem. In such a critical situation tiding over
the crisis with IVIg followed by DCP is a better option. However,
because of cost consideration, most of patients are unable to
afford IVIg in India.
Julian Manzur MD, Havana, Cuba on Nov 16,
I have nothing to add. I agree completely with the thoughts
and doubts of Professor Sontheimer
Nurul Amin MD, Dhaka, Bangladesh on Nov 17,
Pulse therapy with cyclophosphamide & dexamethasone will
be appropriate here. Since denuded areas are generalized then
electrolyte estimation should be done frequently. Systemic antibiotic
with topical antibiotic dressing is also required. More over
close monitoring of the patient is essential.
Samer Ghosn MD, Department of Dermatology and Dermatopathology,
Boston University School of Medicine, Boston, MA, USA on
Dec 6, 2004
Whenever pemphigus vulgaris and erythema multiforme are part
of a differential diagnosis, paraneoplastic pemphigus should
be considered. I believe this patient should be investigated
for a lymphoproliferative condition.