Dermatomyositis is a rare inflammatory myopathy with characteristic skin manifestations and muscular weakness. Polymyositis is a similar disease without skin lesions. Amyopathic dermatomyositis: typical cutaneous manifestation of DM without clinical and/or laboratory findings of muscle involvement for at least 6 months after the onset of skin rash.

1. By
Dr. Saad Raheem Abed

2.  Dermatomyositis is a rare inflammatory
myopathy with characteristic skin
manifestations and muscular weakness.
 Polymyositis is a similar disease without skin
lesions.
 Amyopathic dermatomyositis: typical
cutaneous manifestation of DM without
clinical and/or laboratory findings of muscle
involvement for at least 6 months after the
onset of skin rash.

3.  Dermatomyositis occurs more commonly in female
patients.
 There are 2 types of DM: adult onset type & juvenile type.
 It presents as a proximal symmetrical muscle weakness
with vasculitis affecting the skin, muscles and internal
organs.
 Patients find it hard to raise their arms to comb their hair
or walk up the stairs due to the proximal muscle
weakness.
 It can be severe enough to affect the muscles needed for
speech and swallowing and is also known to cause
respiratory compromise.
 Dysphagia occurrs in as many as 33% of cases.
 Calcinosis can occur in the skin, joints and muscles.

4.  The associated features of PM may precede
by months, accompany, or follow the skin
signs.
 The cutaneous changes sometimes precede
the onset of muscle weakness by more than 1
year.
 The course of adult DM may be acute,
chronic, recurrent, or cyclic.

5.  DERMATOLOGIC MANIFESTATIONS.
 There are six dermatologic features of DM.
 The heliotrope rash and Gottron’s papules are
pathognomonic signs.
 The other features are a photosensitive
violaceous eruption, periungual
telangiectasia, poikiloderma, and scaly red
scalp.

6.  The heliotrope or
"lilac" rash is a
violaceous
eruption on the
upper eyelids and
in rare cases on
the lower eyelids
as well, often with
itching and
swelling (most
specific, though
uncommon)

7. Dermatomyositis. Heliotrope (violaceous) discoloration
around the eyes and periorbital edema

8.  Gottron's
papules. Discrete
erythematous
papules overlying
the metacarpoph
alangeal,
interphalangeal,
elbow or knee
joints.

9. Gottron’s papules, a pathognomonic sign of
dermatomyositis, are round, smooth, violaceous-to-red, flat topped
papules that occur over the knuckles and along the sides of the fingers.
They can be found over bony prominences,
finger, elbows and knees.

10.  Gottron’s sign:
 Confluent macular
violaceous
erythema with or
without oedema in
the same
distribution of
Gttron’s papules.

11. Violaceous scaling patches on the face and
dorsal interphalangeal joints. The knuckles are involved; they
are spared in SLE.

12. Systemic LE. In contrast to dermatomyositis,
erythema and telangiectasia spare the
knuckles.

13.  Centripetal flagellate erythema co
mprises linear, violaceous streaks
on the trunk (possibly caused by
itching pruritic skin).
 Erythematous lesion on the malar region and
the forehead.

14.  Periungual telangiectasias and erythema occu
r
 Mechanic's hands refers to rough, cracked
skin at the tips and lateral aspects of the
fingers forming irregular dirty-appearing
lines that resemble those seen in a laborer.

16. Calcinosis of the skin or muscle is found in
about 50% of children or adolescents with dermatomyositis.
There are firm yellow nodules, often over bony prominences.
They can extrude through the skin and cause infection.

17.  X-ray findings
sometimes include
dystrophic calcificati
ons in the muscles.
 Calcinosis cutis is
usually seen in
juvenile
dermatomyositis, not
adult
dermatomyositis.
 The pain in JDM may
be severe.

18.  Juvenile Dermatomyositis:
 It resembles that seen in adults, except in:
 Calcification occurs more frequently.
 Widespread vasculitis affecting skin, muscles
and GIT (ulceration and hematemesis).
 Low-grade fever is a common symptom.
 Hypertrichosis and lipoatrophy may occur
rarely.
 Malignancy is rare.

19.  Investigation:
 Elevated ESR and CPK, aldolase SGOT along
with typical EMG findings of spontaneous
muscle fibrillation and short polyphasic
muscle potentials.

21.  DIFFERENTIAL DIAGNOSIS.
 A diagnosis of psoriasis might be made if
scale forms on poikilodermatous patches,
especially if there is no photodistribution.
 T-cell lymphoma or lupus might be confused
with poikiloderma.
 Differential diagnoses of early skin lesions
include polymorphic light eruption, contact
dermatitis, and atopic dermatitis.

22.  Treatment:
 This disease has no known cure.
 Specialized exercise therapy may supplement
treatment to enhance quality of life.
 Medications to help relieve symptoms include:
 Prednisolone
 Methotrexate
 Mycophenolate (CellCept / Myfortic)
 Intravenous immunoglobulin
 Azathioprine (Imuran)
 Cyclophosphamide
 Rituximab

23.  Scleroderma is a disease characterized by
sclerosis of the skin and visceral organs,
vasculopathy (Raynaud’s phenomenon), and
the presence of autoantibodies.
 The spectrum of disease is wide, with
systemic and localized forms

25.  Diagnostic criteria(presence of the major one
or 2 of the minor is enough for the diagnosis)
 Major : symmetric cutaneous sclerosis
proximal to metacarpo or metatarso –
phlangeal joints
 Minor
 a- sclerodactyly
 b- digital pitting scars or loss of substance of
digital pads
 c- bibasilar pulmonary fibrosis

26.  A-vascular : altered production and
responsiveness to vasoconstrictive
endothelium, endothelial cell
apoptosis(intimal proliferation and luminal
occlusion), resulting hypoxia leads to
profibrotic cytokines with activation of
fibroblast and increase collagen production,
Reynaud’s phenomenon due to reversible
vasospasm and irrereversible arterial damage.

27.  B- Fibrosis: stimulation of fibroblasts by
cytokines; transforming growth factor β(TGFβ)
activates connective tissue growth factor(CTGF)
leading to stimulation of collagen synthesis → a
matter of increased synthesis rather than
decreased degradation.
 C- Immune activation: presence of antibodies
support role of immune activation e.g. antitopo -
isomerase, anticentromere antibodies against
PDGF.
 It’s Th2 predominent disease with ↑ IL4 (↑ TGFβ,
↑nieve B cells,↓memory B cells),on the other hand
immunosuppressive drugs don’t have major
effect on disease control.

28.  Systemic sclerosis
 the systemic form: diffuse scleroderma and
CREST syndrome.
 The criteria for the diagnosis of scleroderma
are:

29.  Diffuse scleroderma can be rapidly
progressive and potentially fatal;
 there is symmetric fibrous thickening and
hardening (sclerosis) of the skin and fibrous
and degenerative changes in synovium,
digital arteries, and certain internal organs,
most notably the esophagus, intestinal tract,
heart, lungs, and kidneys.

30.  CREST Syndrome
 A more benign, chronic, and localized variant
of scleroderma is called CREST syndrome
(formerly known as acrosclerosis).
 The five clinical features of this disease
(calcinosis cutis, Raynaud’s phenomenon,
esophageal involvement, sclerodactyly, and
telangiectasia).
 Calcinosis is a unique feature of CREST.

31.  In both systemic sclerosis and CREST
syndrome there are three stages of skin
disease: (1)edematous,
 (2) indurative or sclerotic, and (3) atrophic.

32.  Dyspigmentation : very common and not
understood in form of a- diffuse
hyperpigmentation especially areas of
pressure like belt, b- depigmentation in
upper trunk and central face , may both (salt
and pepper).
 Dryness : decreased sweating
 Facial disfigurement (microstomia, retraction
of lips , perioral furrows)
 Cutaneous ulcers(ischemia, fibrotic tissue and
trauma)

33.  Nailfold capillary patterns as detected by
nailfold capillary microscopy may help
distinguish Raynaud’s disease (no
scleroderma) from Raynaud’s phenomenon
(associated with scleroderma).
 A decrease in capillary loops occurs in
Raynaud’s phenomenon.
 This fact may help to predict which cases of
Raynaud’s disease will evolve into systemic
sclerosis.

34.  The telangiectasias of CREST syndrome and
scleroderma have a unique morphology with
telangiectatic matting
 These mats are most commonly found on the
face, lips, palms, and backs of the hands.
 Telangiectasias may be present around the
lips, tongue, and mucous membranes.

35.  Chemically induced scleroderma
 Scleroderma-like diseases can be induced by
a number of
 chemical compounds, such as plastics,
solvents, and drugs.

38. Scleroderma. The hands may be edematous
and swollen early in the disease. These changes progress to
other areas including the face. This edematous stage precedes
the sclerotic stage.

39. The skin is tightly bound down. The fingers are contracted.
Telangiectatic mats are evident on the palms. There are
fingertip ulcerations
Fingertips are narrowed, and the fingers are shortened as a
result of distal bone resorption.

40. Repeated and increasingly severe attacks of
Raynaud’s phenomenon lead to fingertip ulcerations that
leave pitted or star-shaped scars.
Telangiectasias are most obvious in the perioral
area and neck. The skin about the mouth is drawn into furrows
that radiate from the mouth.

43. calcinosis over a joint

49.  PROGNOSIS. Baseline factors that are most
predictive of a poor outcome included the
presence of abnormal cardiopulmonary signs
and abnormal urine sediment (pyuria,
hematuria).
 Subsets of patients with scleroderma with
antibodies to centromere and histone have
severe pulmonary or vascular disease.

50.  CBC (normocytic normochromic anemia)
 Urea and electrolytes
 Urine if proteinuria ,do 24 h urine for protein and
creatinine clearance
 Antibodies (anti scl 70 in diffuse, anti centromere in
limited,ANA +ve in 90%,RH+ IN 30 %)
 Deep skin biopsy.
 CXR (lung disease and changes in cardiac chambers)
 Hand x-ray : ca++ around fingers,erosion and absorption
of tuft of terminal phalanges(acroteolysis)
 Barium swallow (impaired oesophageal motility )
 Upper GI endoscopy
 High resolution C.T.(lung involement ,bibasilar fibrosis)
 Echo,pulmonary function tests