Sclera Derema Info Needed

IMMTEST immtest at
Fri Jul 29 12:55:02 EST 1994

In article <770A3943 at>, ROBB.LIGHTFOOT at (robb lightfoot)

>My 7-year-old niece has a rare disease. It is sclera derma. If the
>disease does not stop, it will turn her into a young version of "the
>elephant man."

>My question is there ANYONE out there knows anything about
>treatments for this. The only treatment offered to my niece was refused
>by my sister. This was Penecillimide D. Apparently this is a very toxic
>substance that could do great damage to my niece, including kidney
>damage and brain damage.

>I'd appreciate the name of any person or school working on this disease
>or on problems with hyper-sensitive immune systems. Any books that you
>can recommend would be apreciated.

Unfortunately, no treatment for scleroderma has proven to be effective. 
Evaluation of the affects of proposed therapeutic agents is usually based
on changes in the skin's texture or tightness, but these indicators are
notoriously difficult to quantify.  More objective measurements, such as
that of pulmonary function in patients with long involvement, are
consistently uninfluenced by any drug therapy.  Among the drugs proposed
are aminobenzoic acid, griseofulvin, penicillamine, and colchicine. 
Neither immunosuppressive nor cytotoxic drugs are effective.

Support measures for certain limited aspects of the disease may be
beneficial.  Raynaud's phenomenon may respond to sympatholytic agents,
such as reserpine, phenoxybenzamine, methyldopa, or guarethidine.  Severe
cases, in which ischemia progresses to gangrene, may require preganglionic
sympathectomy to stop tissue necrosis.  Sympathectomy, however, ususaly
has little effect on chronic, stable Raynaud's phenomenon, and it has not
been shown to influence the eventual course of scleroderma.  Therefore,
this procedure should be reserved for ischemic crises.  Avoiding exposure
to cold temperatures is important in patients with severe Raynaud's
phenomenon.  A warm climate is ideal;  for colder latitudes,
well-insulated gloves (e.g., insulated with goose down) may be helpful. 
Local care of digital ulcerations usually promotes healing.

Esophagitis, esophageal reflux, and stricture are treated by elevating the
head of the patient's bed and by antacids and, if necessary, esophageal
dilatation.  The malabsorption syndrome may sometimes respond to
broad-spectrum antibiotics.

You may consult your local dermatologist or a university dermatology
department.  Dr. Perez of Yale University School of Medicine wrote an
excellent review on the subject.  He may be reached at:

Yale University School of Medicine
Department of Dermatology
LCI 500, P.O. Box 3333
New Haven, CT  06510


Vijay Kumar, Ph.D.
ABMM and ABMLI Diplomate
IMMCO Diagnostics
963 Kenmore Avenue
Buffalo, NY  14223

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