Localized Nodular Myositis/ Focal Myositis
Background Histopathology & Immunohistochemistry Differential Diagnosis
BACKGROUND AND CLINICAL INFORMATION:
Head
Summary: These are two forms of localized inflammatory
myopathy, namely the focal myositis and localized nodular myositis. Their
clinical presentation and histopathology are very similar. The claimed
difference [Smith
AG et al., 2000] is that
focal myositis does not progress to polymyositis but some localized nodular
myositis progress to polymyositis, particularly those with elevated CPK and ESR.
They may well be in the spectrum of a same disease.
Localized nodular myositis presents as solitary mass in young or middle aged
adults with the thigh as the most common site. Patient may or may not have
general symptoms. Clinical features may simulate a soft tissue tumor or even
sarcoma. Histologically, there are multifocal chronic inflammatory cell
infiltration, endomysial inflammatory cell infiltration, myopathic and
regenerating fibers, and fibrosis. Some of them, especially those with elevated
ESR and CPK, may eventually evolve into polymyositis. [Cumming
WJ et al., 1977; Toti
P et al., 1994; Flaisler
F et al., 1993; Smith
AG et al., 2000; Yanmaz
Alnigenis MN et al., 1999]
Focal myositis: Focaly myositis rarely progresses to involve other
muscle but some localized nodular myositis will progress to polymyositis. CPK
and ESR are normal or slightly elevated. The histopathologic features of these
two entities are very similar. It is quite difficult to distinguish these two
entities at the initial presentation. [Toti
P et al., 1994; Flaisler
F et al., 1993; Smith
AG et al., 2000; Yanmaz
Alnigenis MN et al., 1999]
Clinical features:
·
Age and sex: Affect
young and middle-aged adult with no sex predilection.
·
Location: The
thigh and lower leg are the most common locations but the shoulder, arm, back,
and even the tongue have all been affected.
·
Presentation: Localised
nodular myositis usually presents as a painful, sometimes relapsing and
remitting, nodular mass. The growth may be rapid and may clinically suggest a
tumoral or a thrombophlebitic disease.
·
ESR and CPK: They
may or may not be elevated in localized nodular myositis. Focal myositis are
usually not associated with elevated ESR and CPK.
·
EMG: The
three cases of localized nodular myositis described by Cumming
WJ et al., (1977) showed a myopathic pattern.
Prognosis: Localized nodular myositis, particularly cases with
elevated ESR and CPK, may progress to polymyositis. Focal myositis usually does
not progress to polymyositis.
HISTOPATHOLOGY AND IMMUNOHISTOCHEMISTRY:
Head
There
is intense, predominantly interstitial chronic inflammation associated with
scattered foci of muscle fiber destruction and regeneration which resemble
muscle infarcts. Endomysial chronic inflammatory cell infiltration is present.
The lesion may also have substantial fibrosis.
Polymyositis: look for involvement in other muscle.
Other forms of myositis.