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$Unique_ID{BRK04311}
$Pretitle{}
$Title{Vasculitis, Cutaneous Necrotizing}
$Subject{Vasculitis, Cutaneous Necrotizing Dermal Necrotizing Angiitis
Polyarteritis Nodosa Hypersensitivity Vasculitis Wegener's Granulomatosis
Schoenlein-Henoch Purpura Rheumatoid Arthritis Giant-Cell Arteritis Sjogren
Syndrome Discoid Lupus Erythematosus }
$Volume{}
$Log{}
Copyright (C) 1989 National Organization for Rare Disorders, Inc.
698:
Vasculitis, Cutaneous Necrotizing
** IMPORTANT **
It is possible that the main title of the article (Cutaneous Necrotizing
Vasculitis) is not the name you expected. Please check the SYNONYM listing
to find the alternate names and disorder subdivisions covered by this
article.
Synonyms
Dermal Necrotizing Angiitis
Information on the following diseases can be found in the Related
Disorders section of this report:
Polyarteritis Nodosa
Hypersensitivity Vasculitis
Wegener's Granulomatosis
Schoenlein-Henoch Purpura
Rheumatoid Arthritis
Giant-Cell Arteritis
Sjogren Syndrome
Discoid Lupus Erythematosus
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.
Cutaneous Necrotizing Vasculitis is an inflammation of the blood vessel
system which includes the veins, arteries and capillaries. It usually also
affects the skin and may occur alone or in conjunction with allergic,
infectious or rheumatic diseases.
Symptoms
Cutaneous Necrotizing Vasculitis is a common disorder characterized by an
inflammation of the blood vessel walls and skin lesions. These skin lesions
may be flat and red (macules), nodules or hemorrhages under the skin
(purpura). They may occur on many areas of the body but are seen most often
on the back, hands, buttocks, the inside areas of the forearm and the lower
extremities. These skin symptoms may occur only once or at regular
intervals. They will usually last for several weeks and may leave darkened
spots and scarring. In some cases there may be wheel-like lesions that cause
intense itching (urticaria), or ring-shaped lesions and ulcers. Blister-like
lesions (vesicles, bullae) may develop in severe cases. There may also be
fever, generalized discomfort (malaise), muscle or joint pain.
Causes
The exact cause of Cutaneous Necrotizing Vasculitis is unknown. Some lesions
may be caused by an allergic reaction or hypersensitivity to certain
medications such as sulfa or penicillin, other drugs, toxins, and inhaled
environmental irritants. Skin manifestations may also occur because of a
fungal infection, parasites or viral infections, while in some instances the
cause may be due to an autoimmune disorder. Autoimmune disorders are caused
when the body's natural defenses (antibodies, lymphocytes, etc.) against
invading organisms suddenly begin to attack healthy tissue.
Affected Population
Cutaneous Necrotizing Vasculitis affects males and females in equal numbers.
It is a common inflammatory disorder.
Related Disorders
The following diseases can have similarities to Cutaneous Necrotizing
Vasculitis:
Polyarteritis Nodosa is a group of systemic necrotizing vasculitis
including the original classic polyarteritis nodosa, allergic granulomatosis,
and those disorders that have the characteristics of both (an overlap
syndrome). (For more information on this disorder, choose "Polyarteritis" as
your search term in the Rare Disease Database).
Hypersensitivity Vasculitis includes a wide group of vasculitic syndromes
that affect the upper and lower respiratory tract, kidneys and skin. They
are usually caused by an allergic reaction to an unknown antigen.
Wegener's Granulomatosis is a rare collagen vascular disorder that begins
as a localized inflammation of the upper and lower respiratory tract mucosa,
and usually progresses into generalized inflammation of the blood vessels
(vasculitis) and kidneys (glomulonephritis). (For more information on this
disorder, choose "Wegener" as your search term in the Rare Disease Database).
Schoenlein-Henoch Purpura is one of a group of disorders characterized by
purplish or brownish red discolorations of the skin. These spots may be
large or small. Internal bleeding may occur in various areas of the body.
This blood vessel disorder may affect the skin, joints, gastrointestinal
system, kidneys, and in a very few cases the central nervous system. Little
is known about the cause of this form of purpura although it may be an
allergic reaction which more often occurs in children than adults. (For more
information on this disorder, choose "Schoenlein" as your search term in the
Rare Disease Database).
Rheumatoid Arthritis is a common autoimmune disease that affects the
joints. The exact cause is unknown. It is characterized by a loss of
appetite, extreme fatigue and joint pain with deformities. The location of
painful joints may change place (migration) and often more than one joint is
affected. Pain, early morning stiffness, aching joints chiefly in the hands,
knees, feet, jaws, and spine occur. Once affected, a joint may remain
painful for a long time and eventually become deformed. (For more
information on this disorder, choose "Rheumatoid Arthritis" as your search
term in the Rare Disease Database.
Giant-Cell Arteritis is a chronic inflammatory disease of the branches of
the aortic arch. This disorder is found principally in the temporal and
occipital arteries, but may develop in almost any of the large arteries. (For
more information on this disorder, choose "Giant Cell" as your search term in
the Rare Disease Database.)
Sjogren Syndrome is an autoimmune disorder causing degeneration of the
tear and saliva glands. It is often associated with arthritis. Patients
often complain of a gritty, burning sensation in their eyes due to loss of
lubrication. When their mouths become dry, chewing and swallowing food is
difficult. The lack of saliva causes particles of food to stick to the
cheeks, gums, and throat. Other symptoms include a weak voice and dental
decay, plus dryness of the nose, skin and vagina. (For more information on
this disorder, choose "Sjogren" as your search term in the Rare Disease
Database.)
Discoid Lupus Erythematosus is a chronic and recurrent autoimmune
disorder primarily affecting the skin. It is characterized by sharply
circumscribed spots (macules) and plaques displaying redness (erythema),
plugging of follicles, scales, vascular lesions (telangiectasia), and wasting
(atrophy). There are two varieties: one with lesions above the chin, the
other with or without facial involvement but causing skin lesions on the rest
of the body. (For more information on this disorder, choose "Lupus" as your
search term in the Rare Disease Database.)
Therapies: Standard
Treatment of Cutaneous Necrotizing Vasculitis depends on the cause and
symptoms. Removing the irritating agent (e.g., drug) and treating the
underlying infection will usually eliminate the symptoms of this disorder.
The drugs prednisone, cyclophosphamide, pentoxifylline and azathioprine have
proven to be successful in treating the autoimmune form of Vasculitis. Other
treatment is symptomatic and supportive.
Therapies: Investigational
At the present time research is being conducted on the use of high dose
intravenous gammaglobulin for some forms of Cutaneous Vasculitis.
Plasmapheresis may be of benefit in some cases. This procedure is a method
for removing unwanted substances (toxins, metabolic substances and plasma
parts) from the blood. Blood is removed from the patient and blood cells are
separated from plasma. The patient's plasma is then replaced with other
human plasma and the blood is retransfused into the patient. This therapy is
still under investigation to analyze side effects and effectiveness. More
research must be conducted to determine long-term safety and effectiveness of
these treatments.
This disease entry is based upon medical information available through
November 1989. 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 Cutaneous Necrotizing Vasculitis, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
NIH/National Heart, Lung and Blood Institute
9000 Rockville Pike
Bethesda, MD 20892
(301) 421-8453
References
INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and
Co., 1987. Pp. 1366-1368.
PULMONARY DISEASES AND DISORDERS, Volume 2, 2nd Ed.: Alfred P. Fishman
M.D. ed.-in-chief; McGraw-Hill Book Co., 1980. Pp. 381-384.
THE MERCK MANUAL, Volume 2, 14th Ed.: Robert Berkow, M.D. ed.-in-chief;
Merck, Sharp & Dohme Laboratories., 1982. Pp. 798
DIAGNOSTIC STUDIES FOR SYSTEMIC NECROTIZING VASCULITIS. SENSITIVITY,
SPECIFICITY, AND PREDICTIVE VALUE IN PATIENTS WITH MULTISYSTEM DISEASE. P.J.
Dahlberg et al.; ARCH INTERN MED, (January 1989; issue 149 (1)). Pp. 161-
165.
SEVERE LEUKOCYTOCLASTIC VASCULITIS OF THE SKIN IN A PATIENT WITH
ESSENTIAL MIXED CRYOGLOBULINEMIA TREATED WITH HIGH-DOSE GAMMA-GLOBULIN
INTRAVENOUSLY. B.W. Boom et al.; ARCH DERMATOL, (October 1988; issue 124
(10)). Pp. 1550.
URTICARIAL VASCULITIS PROGRESSING TO SYSTEMIC LUPUS ERYTHEMATOSUS. E.
Bisaccia et al.; ARCH DERMATOL, (July 1988; issue 124 (7)). Pp. 1088-1090.
LIVEDO VASCULITIS. THERAPY WITH PENTOXIFYLLINE. W. Sams. ARCH DERMATOL,
(May 1988; issue 124 (5)). Pp. 684-687.