$Unique_ID{BRK03725}
$Pretitle{}
$Title{Erythromelalgia}
$Subject{Erythromelalgia Mitchell Disease Weir Mitchell Syndrome Gerhardt
Disease Primary Erythromelalgia Secondary Erythromelalgia}
$Volume{}
$Log{}

Copyright (C) 1986, 1987, 1988, 1989 National Organization for Rare Disorders,
Inc.

245:
Erythromelalgia

** IMPORTANT **
It is possible the main title of the article (Erythromelalgia) is not the
name you expected.  Please check the SYNONYMS listing to find the alternate
names and disorder subdivisions covered by this article.

Synonyms

     Mitchell Disease
     Weir Mitchell Syndrome
     Gerhardt Disease

DISORDER SUBDIVISIONS

     Primary Erythromelalgia
     Secondary Erythromelalgia

General Discussion

** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only.  It should not be used for diagnostic or treatment
purposes.  If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.


Erythromelalgia is a syndrome of sudden intensive spastic dilation of
blood vessels (paroxysmal vasodilation).  This causes burning pain, increased
skin temperature, and redness of the feet and, less often, the hands.

Symptoms

Erythromelalgia is characterized by attacks of burning pain in hot red feet
or hands.  Increase of symptoms is often triggered by hot weather.  Symptoms
may remain mild for years or they may become so severe that total disability
can result.

Causes

Primary Erythromelalgia may be transmitted as a dominant hereditary disorder.
(Human traits including the classic genetic diseases, are the product of the
interaction of two genes for that condition, one received from the father and
one from the mother.  In dominant disorders, a single copy of the disease
gene (received from either the mother or father) will be expressed
"dominating" the normal gene and resulting in appearance of the disease.  The
risk of transmitting the disorder from affected parent to offspring is 50%
for each pregnancy regardless of the sex of the resulting child.)

Secondary Erythromelalgia may occur in patients with proliferative
disorders of the bone marrow, hypertension, impairment of the return of
venous blood from the legs (venous insufficiency), diabetus mellitus, or
polycythemia vera.

Affected Population

Males and females are affected in equal numbers.

Related Disorders

Causalgia syndrome or Traumatic Erythromelalgia is characterized by a
persistent diffuse burning pain, especially in the palms of the hands and the
soles of the feet.  It is aggravated by minimal physical stimuli such as
friction and heat.

Therapies:  Standard

Attacks of Erythromelalgia can sometimes be avoided or reduced by rest,
elevation of the extremity, and cold applications to the affected area.
Correction of the underlying disease in secondary forms of Erythromelalgia is
indicated.  In Primary Erythromelalgia modest doses of aspirin may produce
relief, but this therapy is not always successful.

Avoiding factors that produce vasodilation is usually helpful.  The use
of ephedrine, propranolol or methysergide may also produce relief in some
patients.

Therapies:  Investigational

Sodium nitroprusside (sodium ferri cyanide) has been used intravenously with
some success on Erythromelalgia.  It is a potent antihypertensive drug that
can cause serious side effects.  More research is necessary to determine the
safety and effectiveness of this drug for Erythromelalgia.

This disease entry is based upon medical information available through
June 1988.  Since NORD's resources are limited, it is not possible to keep
every entry in the Rare Disease Database completely current and accurate.
Please check with the agencies listed in the Resources section for the most
current information about this disorder.

Resources

For more information on Erythromelalgia, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     (203) 746-6518

     Erythromelalgia Association of America
     Good Samaritan Hospital & Medical Center
     1015 NW 22nd Avenue
     Portland, OR  97210

For information on genetics and genetic counseling referrals, please
contact:

     March of Dimes Birth Defects Foundation
     1275 Mamaroneck Avenue
     White Plains, NY 10605
     (914) 428-7100

     Alliance of Genetic Support Groups
     35 Wisconsin Circle, Suite 440
     Chevy Chase, MD  20815
     (800) 336-GENE
     (301) 652-5553

References

SODIUM NITROPRUSSIDE TREATMENT IN ERYTHROMELALGIA:  S. Ozsoylu, et al.;
European Journal Pediatr (1984:  issue 141).  Pp. 185-187.