Systemic                                                Sclerosis
--------------------------------------------------

I saw a woman this week with systemic sclerosis  (Scl-70+,  renal
failure)  originally  diagnosed  in  4/95.   She has diffuse skin
involvement  and,  to  date,  has  been  treated  only  with   d-
penicillamine.  This was stopped with the onset of renal failure.
She came to me with  a  several  month  history  of  a  pruritic,
papular eruption on both forearms.  Without obvious precipitating
cause, she developed numerous papules on  both  forearms  over  a
very short period of time.  These were diagnosed on the "outside"
as warts and responded (by her history) to treatment with topical
steroids by vesiculating.

On exam, there is diffuse cutaneous  sclerosis,  hyperpigmention,
sclerodactaly and a few small ulcers on the digits.  Her forearms
are covered from elbow to wrist with coalescing, tense, verrucous
papules.  They appear shiny and edematous, but there are no frank
vesicles.

A biopsy is pending, but I expect to see lymphangiectasis.  While
this has been reported in the literature (thank you, Drs. Provost
and Tuffanelli), this doesn't seem to be an entity well known  to
many  rheumatologists/dermatologists.   I  haven't  seen  such an
impressive case...or is  my  lack  of  grey  hair  showing?   I'm
intrigued         about        the        distribution,        in
rheumatologists/dermatologists.    I   haven't   seen   such   an
impressive  case...or  is  my  lack  of  grey  hair showing?  I'm
intrigued about the distribution, in particular, the  sparing  of
the legs. I don't know what her fluid status has been, so I don't
know to what extent this might have contributed.  Any  experience
with this condition, or other comments?  I'm about to offer her a
trial of PUVA.

Kathy Schwarzenberger, M.D.  --------------------------


I'd like to see if there is any sign of  pemphigus  -  especially
with  the  patient's present Rx.  The other differential would be
multiple eruptive KA's, and even Sweet's Syndrome

Robert I. Rudolph, M.D.  --------------------


I have seen a patient  here  at  Emory  several  years  ago  with
scleroderma   who   developed  innumerable  fibrotic  dome-shaped
papules over much of her skin surface. Biopsies  repeatedly  were
unrevealing  (don't  remember the path, but I think it was mainly
increased  collagen)  and  Grand  Rounds  presentation  was  also
unhelpful.  Don't  know  what  ever  happened  to  her:  I'll ask
around.

Mark Ling, M.D., Ph.D.  ------------------


I have seen and have reported renal failure  patients  developing
perforating folliculitis.  Have these papules been biopsied?

Jeff Marmelzat, M.D.  ------------------


I'm not sure what she has, but I'bve observed that  PSS  and  EMS
patients  get  very  funny  manifestations  of cutaneous edema in
their  sclerotic  skin.  For  exp.  venous  insufficiency  in   a
sclerodermatous  leg sometimes presents as blistering rather than
edema.  I'd  consider  a   contact   derm   or   some   localized
irritation/infection as a first guess.

Guy Webster -----------


I have seen a patient  here  at  Emory  several  years  ago  with
scleroderma   who   developed  innumerable  fibrotic  dome-shaped
papules over much of her skin surface. Biopsies  repeatedly  were
unrevealing  (don't  remember the path, but I think it was mainly
increased  collagen)  and  Grand  Rounds  presentation  was  also
unhelpful.  Don't  know  what  ever  happened  to  her:  I'll ask
around.

Mark Ling, M.D., Ph.D.  ------------------


Could  it  be  a  case  of  scleromyxoedema  or  a   combination?
Circulating   paraprotein?    In   scleromyxoedema  you  have  an
extensive proliferation of fibroblast throughout the  dermis  and
deposit  of  abundant  mucin  in the reticular dermis.  There are
some articles  pointing  out  scleromyxoedema  with  features  of
systemic sclerosis and one (below) with the combination.

(Papular   mucinosis   (scleromyxoedema)   complicating   diffuse
systemic  sclerosis:clinical  features  and  electron  microscope
observations.   Varga  J  et  al.    Br   J   Rheumatol,   31:11,
1992Nov,779-82)

Peter Gisslen M.D ----------------


Great minds think alike...I just posted the same sort of message.
The  edema-blister PSS and EMS patients of which I spoke were not
scleromyxedema however.  I've a second thought about  papules  in
PSS...  perhaps  the  patient actually has scleromyxedema/papular
mucinosis.

Guy Webster -----------