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Dermatomyositis: Dermatoses Morphology Etiology Pathogenesis

Dermatomyositis is an inflammatory myopathy characterized by skin and muscle involvement. It presents with a reddish-purple rash over the eyelids, knuckles, elbows, knees and shoulders. Muscle weakness typically involves the shoulder and pelvic girdle. Treatment involves high-dose corticosteroids. Risk factors for poor prognosis include failure to induce remission, high white blood cell count, fever and older age at diagnosis.

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Riena Austine Leonor Narcilla
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65 views

Dermatomyositis: Dermatoses Morphology Etiology Pathogenesis

Dermatomyositis is an inflammatory myopathy characterized by skin and muscle involvement. It presents with a reddish-purple rash over the eyelids, knuckles, elbows, knees and shoulders. Muscle weakness typically involves the shoulder and pelvic girdle. Treatment involves high-dose corticosteroids. Risk factors for poor prognosis include failure to induce remission, high white blood cell count, fever and older age at diagnosis.

Uploaded by

Riena Austine Leonor Narcilla
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
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Download as PDF, TXT or read online on Scribd
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Dermatoses Morphology Etiology Pathogenesis Treatment/Management

DERMATOMYOSITIS -Predilection sites: nape of the neck, -F>M; common in blacks; relatively rare -Prednisone: the mainstay
upper chest (V) pattern, and upper back, of acute treatment (dooses
neck, and shoulder (shawl) pattern -Evolve through multiple sequential phases: beginning with
1) A genetically determined susceptibility phase 1mg/kg/day until the
2) An induction phase triggered by an environmental severity decreases and
stimulus (eg. Ultraviolet light, infection) muscle enzymes are
involving loss of tolerance to self-antigens in skin almost normal)
and skeletal muscle
3) An autoimmune expansion phase -Methotrexate and
4) An injury phase involving multiple immunologic mycophenolate mofetil
effector mechanisms are used as steroid-sparing
-Edema and pink violet discoloration are agents
often signs of inflammation in the -Characterized by inflammatory myositis and skin
underlying striated orbicularis oculi disease -Daily use of sunscreen
muscle (Heliotrope rash) with high SPF
-Variant:
a) Amyopathic DM: subclinical or absent -Antimalarials, such as
myopathy hydroxychloroquin are
-Telangiectatic vessels often become b) Polymyositis PM: muscle involvement without useful in abating the
prominent in the proximal nailfolds skin changes eruption of DM

-Enlarge capillaries of the nailfold as -Muscle involvement: weakness of proximal muscle


dilated, sausage-shaped loops with groups in characteristic Prognosis
adjacent avascular regions
Diagnostic criteria -Major causes of death are
cancer, ischemic heart
Pathognomonic:  Dermatomyositis: Patients with the disease, and lung disease
CUTANEOUS lesion + 4 remaining
-Gottron’s sign: pink to reddish-purple  Polymyositis: Patients with NO CUTANEUS -Independent risk factors
atrophic or scaling eruption often over lesion > or = 4 remaaining  Failure to induce
the knuckles, knees, and elbows clinical remission
 Cutaneous lesions (erythema, scaling, swelling)  White cell count
- Gottron’s papules: flat-topped,  Heliotrope rash: pinking violet edematous above 10,000/
polygonal, violaceous papules over the erythema on the upper palpebral mm3
knuckles; less common but are highly  Gottron’s papules or sign: pinkish violet flat-  Temperature
characteristic of DM topped papules, atrophy, or erythema on the greater than 38
extensor surfaces and finger joints degree Celsius at
 Proximal muscle weakness (upper or lower diagnosis
extremity and trunk)  Older age
 Muscle pain on grasping or spontaneous pain  Shorter disease
 Myogenic changes on EMG history
-Mechanic’s hand  Elevated serum creatine kinase or aldolase level  Dysphagia
 Hyperkeratosis, scaling,  Positive anti-Jo-1 (histadyl tRNA synthetase)
fissuring, hyperpigmentation antibody -Early aggressive therapy
over the fingertips, side of the  Non-destructive arthritis or arthralgia in juvenile cases is
thumb, and fingers with  Systemic inflammatory signs (fever, elevated associated with a lower
occasional involvement of the serum CRP level or accelerated ESR) incidence of disabling
palms  Pathologic findings compatible with inflammatory calcinosis cutis
 Seen in 70% of patients with myositis
with antisynthetase antibodies
Cutaneous lesions
 Pruritic scaly pink patches are often seen in
Amyopathic DM
 Pruritus may be severe in some cases and is
much more common in DM than in psoriasis or
LE
 Bullous DM may portend a poor prognosis (often
with severe inflammatory myopathy or lung
disease)

Muscle involvement
 Skin involvement commonly precedes muscle
involvement but some patients have typical skin
findings of DM but never develop clinically
apparent muscle involvement
 Muscle weakness is seen symmetrically involving
the shoulder girdle, pelvic regions, and hands
 Difficulty in lifting even the lightest object,
unable to raise their arms to comb their hair and
rising from a chair may be impossible without
“pushing off” with the arms
 Pain in the legs when standing barefoot or of
being unable to climb stairs
 Difficulty in swallowing, talking, and breathing
 Some patients with severe diaphragmatic disease
require mechanical ventilation and cardiac failure
may be present in the terminal phase of the
disease
 Calcium deposits in the skin and muscle: 50% in
children with DM
 Calcification is related to duration of disease
activity and severity
 Calcinosis of the dermis, subcutaneous tissue, and
muscle occur mostly on the upper half of the body
around the shoulder girdle, elbows, and hands

-DM may overlap with other connective tissue diseases

-DM is associated with malignancy with highest


probability within 2 years of the diagnosis; 50 -60 years of
age

-Factors associated with malignancy include age,


constitutional symptoms, rapid onset of DM, the lack of
Raynaud phenomenon, and a grossly elevated ESR or
creatine kinase level

Laboratories
 Elevated serum levels of creatine kinase
 Other indicators of active muscle disease:
aldolase, lactic dehydrogenase and transaminases
 Laboratories: Leukocytosis, anemia with low
serum iron and an increased ESR
 Positive ANA test are seen in 60-80%; 35-40%
have myositis specific antibodies

1. Childhood DM Two childhood variants

1) Brunsting type: more common, has a slow


course, progressive weakness, calcinosis, and
steroid responsiveness
2) Banker type: characterized by a vasculitis of the
muscles and gastrointestinal tract, rapid onset of
severe weakness, steroid unresponsiveness, and a
high death rate

-Internal malignancy is seldom seen in children

-Calcinosis cutis is more common in children with severe


diseases

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