| Skin
Diseases Similar to Scleroderma (Main Menu) |
|
|
| Eosinophilic
Fasciitis (EF) or Shulman's Syndrome |
| This page
was written by Shelley
Ensz and has not yet been medically edited. The debate
is ongoing as to whether or not eosinophilic fasciitis is a
form of scleroderma.
See disclaimer. |
|
| What is
Eosinophilic Fasciitis (Shulman's Syndrome)? |
| Eosinophilic
Fasciitis is a scleroderma-like disorder characterized
by symmetric and painful inflammation, swelling, and induration
involving arms and legs but not the most acral areas involved
in scleroderma. Merck. |
| Eosinophilic
Fasciitis is a syndrome characterized by tenderness and
swelling of the extremities caused by inflammation of the
fascia and muscles. Henry Ford Health System. |
| Eosinophilic
Fasciitis & Eosinophilia-Myalgia Syndrome Diagnosis
is made by looking at a sample (biopsy) of affected skin.
It is thought that EF is a type of scleroderma. About.com
01/18/04. (Also see: Eosinophilia-Myalgia) |
| First
time eosinophilic fasciitis (EF) was described by Shulman. Eosinophilic
fasciitis is classified to wide spectrum of scleroderma disorders
by many authors. PubMed. Przegl Lek. 2003;60(2):116-9. |
| Scleroderma and Eosinophilic
Fasciitis |
| Eosinophilic
fasciitis—current database. For the first time
eosinophilic fasciitis (EF) was described by Shulman. It
occurs predominantly in young men, rarely in older women
and sporadically in children. Eosinophilic fasciitis is classified
into the wide spectrum of scleroderma disorders by many authors. PubMed.
Pol Merkuriusz Lek. 2004 Jun;16(96):585-8. |
| Eosinophilic
Fasciitis Since 1974, more than 200 patients with EF
have been reported, and debates still are ongoing as to whether
EF represents a variant of scleroderma. eMedicine. |
| Symptoms of Eosinophilic
Fasciitis |
| Eosinophilic
fasciitis (EF) associated with autoimmune thyroiditis. EF
is frequently associated with hematological abnormalities,
including malignant lymphoproliferative diseases, but rarely
associated with autoimmune thyroiditis. In the present study
we report a case of eosinophilic fasciitis associated with
autoimmune thyroiditis. PubMed. Korean J Intern Med. 2005
Jun;20(2):180-2. (Also see: Autoimmune
Thyroiditis) |
| Eosinophilic
Fasciitis: Symptoms The initial features are pain, swelling,
and inflammation of the skin, followed by induration, creating
a characteristic orange-peel configuration most evident over
the anterior surfaces of the extremities. Merck. |
| Eosinophilic
fasciitis: analysis of a series of 10 patients. Eosinophilic
fasciitis does not always have a good prognosis. Ventilatory
restriction, perimyositis and poor treatment response are
not infrequent in a series of patients attended in a general
hospital. PubMed. Med Clin (Barc). 2005 Jun 25;125(4):145-8. |
| Diagnosis of Eosinophilic
Fasciitis (Shulman's Syndrome) |
| Eosinophilic
fasciitis 30 years after - what do we really know? Report
of 11 patients and review of the literature. The diagnosis
of EF can be established by clinical, laboratory and histological
findings. In general, corticosteroids are highly effective
in EF and only a minority of patients need other immunosuppressive
or cytostatic drugs. PubMed. Dermatology. 2006;213(2):93-101. |
| An
unusual case of generalized oedema. The biopsy confirmed
the diagnosis of eosinophilic fasciitis. Clinical and MRI
findings suggested an early stage of disease and the patient
was treated with low-dose corticosteroids. PubMed. Acta
Clin Belg. 2003 May-Jun;58(3):201-4. |
| The
diagnostic approach when confronted with a pseudo-tumoral
lesion and fever is difficult since it evokes an infectious,
rheumatismal inflammatory or even systemic disease. It
is important to rule out its differential diagnosis from
local edematous scleroderma, which does not share the same
prognosis. PubMed. Presse Med 2003 Mar 22;32(11):498-500. |
| Eosinophilic
fasciitis and eosinophilic cellulitis in a patient with abnormal
circulating clonal T cells: Increased production of interleukin
5 and inhibition by interferon alfa. The coexistence
of eosinophilic fasciitis and cellulitis in a patient with
an abnormal circulating T-cell clone and increased IL-5 production
are unique and might be responsible for the eosinophilia
and eosinophil-mediated tissue injury. PubMed. J Am Acad
Dermatol. 2003 Dec;49(6):1170-4. |
| Research About Eosinophilic
Fasciitis (Shulman's Syndrome) |
| Eosinophilic Fasciitis (EF): Clinical, Biological, Histological and Therapeutic Features from a Series of 28 Patients. EF is a rare disease characterized by cutaneous and muscular symptoms, a frequent blood eosinophilia. The mainstay treatment is steroids alone or in combination with an immunosuppressive drug, mainly methotrexate, allowing a satisfying response in more than 80% of patients. Damien Sène. 1630/244. ACR 2007. |
| Lichen
sclerosus and eosinophilic fasciitis as manifestations of
chronic graft-versus-host disease: expanding the sclerodermoid
spectrum. We describe 6 patients, all of whom developed
LS and two of whom also developed EF in the context of chronic
GVHD. Although all patients eventually developed morpheaform
and/or sclerodermoid GVHD, LS was a prominent part of the
initial presentation of chronic cutaneous GVHD in every case. PubMed.
J Am Acad Dermatol. 2005 Oct;53(4):591-601. (Also see: Lichen
Sclerosus and GVHD) |
| Eosinophilic
fasciitis successfully treated with cyclosporine. We
report on a 45-year-old female who developed eosinophilic
fasciitis. High-dose systemic corticosteroid therapy was
ineffective, but cyclosporine treatment led to remission. PubMed.
Clin Rheumatol. 2005 Apr 29. |
| Miliary
tuberculosis in a patient with eosinophilic fasciitis. This
is the first reported case of miliary tuberculosis associated
with eosinophilic fasciitis. PubMed. South Med J. 2005
Jan;98(1):107-10. |
| Eosinophilic
fasciitis associated with Borrelia afzelii infection. We
treated the patient with prednisolone, doxycycline and amoxicillin,
which alleviated the symptoms. This is the first report to
show that Borrelia afzelii was involved in eosinophilic fasciitis. PubMed.
Rinsho Shinkeigaku. 2004 Apr-May;44(4-5):299-302. |
| In
Situ Immunophenotype of the Inflammatory Infiltrate in Eosinophilic
Fasciitis. Some of these CD8+ lymphocytes contain granzyme
B, thus suggesting a cytotoxic cellular immune response in
EF, which could be triggered by infectious or environmental
agents. J Rheumatol NO. 8 AUGUST 2003;30:1811-5. |
| Personal Stories of
Eosinophilic Fasciitis (Shulman's Syndrome) |
| Beth
R: Eosinophilic Fasciitis My arms and legs are still
tight and I still cannot make a fist... |
| Claude:
Eosinophilic Fasciitis I am a forty-four-year-old
male from Ottawa, Canada. In the late spring, early summer
of 2003, my wife noticed some red splotches on my lower legs... |
| Gerry
Fitzpatrick: Eosinophilic Fasciitis My hands have
become very weak and with all my other medical problems,
this one is really depressing me the most... |
| Gillian:
Eosinophilia-Myalgia Syndrome or Eosinophilic Fasciitis My
arms and legs still feel really hard and are very inflexible.
I can't fully open my hands or point my toes... |
| Henry:
Eosinophilic Fasciitis The disease then grabbed hold
and started spreading like wild fire... |
| Jennifer
Foster: Eosinophilic Fasciitis (Ontario, Canada) My
husband has been extraordinarily accepting of the rapid deterioration
of my once charming and petite figure... |
| Jessica
N: Eosinophilic Fasciitis Sometimes I still worry
about me and how my parents are dealing with this... |
| John:
Eosinophilic Fasciitis or Scleroderma-Like Condition I
have knee contractures that prevent me from walking, very
tight skin in the legs... |
| Jon T: Eosinophilic Fasciitis I am stumped as to the possibility for me
to return
to normal duties at work, and what the normal prognosis for someone with this
disease can expect? |
| Kathleen
S: Eosinophilic Fasciitis I noticed that winter during
gym, that when we did push-ups, I had to do knuckle push-ups
because I could not straighten my fingers... |
| Karen
H: Eosinophilic Fasciitis I am experiencing extreme
fatigue and muscle pain on a daily basis... |
| Keith
H: Eosinophilic Fasciitis (EF) I have a rare skin
disease called eosinophilic fascitiis, a skin and muscle
disease which causes tightness of the skin, and sometimes
a reddish coloration of the skin in areas... |
| Lindsey:
Eosinophilic Fasciitis (EF) My skin remains very thick
and constricted... |
| Lori
S: Mother of Eosinophilic Fasciitis Patient On the
second biopsy, they went further into the fascia and confirmed
the diagnosis of eosinophilic fasciitis (EF). I would like
to talk to other parents or patients with EF... |
| Susan
H: Eosinophilic Fasciitis While I was in the hospital
and starting dialysis, my right arm became hard as a rock
and swollen... |
