Rick Sontheimer MD, Professor and Vice-Chairman,
Dept. of Dermatology University of Oklahoma Health Sciences
Center, Oklahama City,OK, USA on May 26, 2006
Might consider a trial of antimalarials (hydroxychloroquine
6.5 mg/kg/day lean body mass and if that fails add quinacrine
100 mg/day) as a systemic immunosuppressive sparing agent
David Elpern MD, Williamstown, MA, USA on
March 27, 2006
This patient would probably do very well if he were compliant.
There are strong cultural factors which prevent this from the
sounds of it. Does he want to live? At age 80, is he ready to
die? If he came in for regular visits, he might be maintained
with low doses of prednisone and doxycycline or dapsone. I have
a similar case who has done well with 100 mg of dapsone a day
for the past ten years after a month or so of prednisone. Dapsone,
in an 80 yo non-compliant patient could be hazardous, however.
IVIg is a very expensive proposition and one wonders if it is
necessary for most uncomplicated cases of pemphigus. This case
highlights the difficulties of treating such a disease process
in patients who do not buy into our therapeutic paradigm.
Khalifa Shaquie MD, PhD, Professor of Dermatology,
College of Medicine, University of Baghdad, Baghdad, Iraq
on March 29, 2006
There are no features of paraneoplastic pemphigus and the picture
is in favour of pemphigus vulgaris but there is no oral involvement.
The management is by oral prednisolone plus azathioprine. Dapsone
could be added.
Henry Foong FRCP Ipoh, Malaysia on March 29,
2006
Paraneoplastic pemphigus (PNP) is characterised by a polymorphic
eruption with severe mucosal involvement which is not present
in this patient. Histology typically shows suprabasal clefting,
prominent vacuolar changes of the basal cells and a lichenoid
infiltration. Direct immunofluorescence shows the typical intercellular
deposition of IgG and complement.
The first-line therapy for treatment of PF is a systemic corticosteroid.
Immunosuppressive drugs such as azathioprine and dapsone may
be used as adjuvants to corticosteroids but hydroxychloroquine,
cyclophosphamide, mycophenolate mofetil, and methotrexate or
even immunomodulators such as tetracyclines and niacinamide
are possible alternatives.
Due to exorbitant costs, I would reserve IVIg for severe widespread
PF not responding to oral corticosteroids and adjuvant therapy.
Thamir Alkubaisi MD, Baghdad, Iraq, on June
2, 2006
Thank you Dr. Hussain Mahdi, with much thanks for VGRD for
this interesting case. Prednisolone and azathioprine (as steroid
sparing drug) can be used until improvement in clinical picture
and reduction in serum Antibodies depending on IIF.
Haitham Alqari MD, Bahrain on June 2, 2006
Nice case. Clinically the pictures are strongly suggestive
of pemphigus vulgaris. No feature of pemphigus foliaceus at
all. Paraneoplastic pemphigus presents typically with prominent
mucosal involvement especially lips. I will consider repeating
skin biopsy for histo and immunofluorescence. With regard to
treatment I want consider IVIG at first because of the cost
+ pemphigus vulgaris responds very well to systemic steroid
and other modalities.
Abbas Alshammari MD, Doha, Qatar on June 5,
2006
Clinically,the case is compatabe with PF in the absence of
oral involvment. So is IF. The histopathology seems to me a
subcorneal blister of secondary infection full of PMNs plus
secondary acantholysis. The idea behind presenting this case
is to demonstrate the role of IVIG in resistant cases of pemphigus.
1.The case showed good respons to topicals used and oral prednisolone
and the author has many choices before considering it as a resistant
case.
2.The goal of treating pemphigus is not how to induce a rapid
remission but to maintain a good control with a safest medicine
considering costing.
3.The author didn't show what is next after a 4 days of IVIG
therapy or follow up period.
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