semoga membantu. dan kia saling berbagi.

1. Dermatomyositis

2. • Dermatomiositis (DM) adalah penyakit autoimun sistemik dengan
distribusi bimodal yang menyerang anak-anak dan orang dewasa,
ditandai dengan peradangan dan kerusakan pada kulit dan otot.
• Penyakit paru interstitial (ILD) mempengaruhi 20% pasien dan
merupakan sumber utama morbiditas dan mortalitas pada pasien
ini.Pada orang dewasa, DM menandai diagnosis keganasan internal
yang hidup bersama pada 10% hingga 20% kasus.
• Tinjauan menyeluruh dari sistem dan pemeriksaan keganasan,
termasuk pemindaian tomografi terkomputasi pada dada, perut, dan
panggul, mungkin bijaksana untuk mendeteksi kanker tersembunyi
yang terlewatkan pada skrining rutin yang sesuai dengan usia.

4. CLINICAL FEATURES
• Triggers may be present such as substantial ultraviolet exposure,
strenuous activity (for the patient with concurrent myositis), or recent
malignancy
• Sering ada pruritus signifikan yang berhubungan dengan kulit yang
terkena, terutama pada kulit kepala, yang juga dapat digambarkan
sebagai “tightness" atau rasa terbakar atau dengan kualitas
dysesthetic lainnya seperti merangkak atau kesemutan.

5. • The natural history of the eruption is chronic and often
progressive, ultimately resulting in a background of
dyspigmentation, atrophy, and telangiectasias.
• The scalp is one of the most commonly involved sites, often
only with pink to red erythema, but there may be associated
fine white scale.
• The scalp can be affected in any location but often involves a
linear band just above and below the frontal hairline.

6. • The heliotrope sign may exemplify the pink to purple violet hue of the
eruption color of the flower petals after which the sign is named
• erythema of the lateral canthi, medial canthi, and adjacent nasal
sidewalls can be seen

7. • Trunk involvement is often seen on the posterior neck, upper back,
and shoulders, known as the shawl sign
• Patients can display symmetric violaceous erythema, often with a
livedo character, symmetrically on the lateral lower back.

8. • Confluent violaceous erythema
on the sun-exposed areas of
the lower anterior neck and
anterior chest is termed the V-
neck sign

9. • The violaceous to pink papules over the IP and MCP joints are termed
Gottron papules

10. They may display the same range of features
seen elsewhere
• such as poikiloderma, atrophy, hypopigmentation,
hyperkeratosis, or ulceration
• Sebagai catatan, banyak tempat dari area keterlibatan kulit
DM kutaneus tidak harus berada di area paparan ultraviolet
(UV) (disebut “photodistributed”), termasuk kulit kepala,
punggung bawah, dan paha lateral. Beberapa pasien dapat
datang dengan erupsi fotodistribusi klasik, tetapi lebih sering
eritema tidak merata bahkan di tempat dengan risiko tinggi
paparan sinar UV.

13. • Other characteristic hand findings include the, socalled “mechanic’s”
hands
• This finding was initially described as hyperkeratosis and fissuring
along the medial thumb and lateral second and third digits

14. • Visualization of the oral mucosa,
particularly the hard palate, may
provide a valuable sign to aid in the
diagnosis of DM. One can observe a
symmetric violaceous patch across the
midline of the hard palate, termed the
ovoid palatal patch

17. • Kerusakan kulit akibat DM dicerminkan oleh bercak-bercak
hiperpigmentasi pasca inflamasi berwarna coklat, sering kali
berretikulat, di area aktivitas penyakit sebelumnya.
• Aktivitas penyakit yang berlangsung lama, biasanya di daerah
yang terpapar sinar matahari, menghasilkan kerusakan yang
lebih signifikan, ditandai dengan atrofi, hipopigmentasi,
hiperpigmentasi, dan telangiektasis, yang disebut
poikiloderma.

18. • Poikiloderma is a late manifestation and is not diagnostically
specific because many other acquired and congenital
diseases result in poikiloderma such as
• cutaneous lupus,
• chronic actinic damage (poikiloderma of Civatte),
• poikilodermatous mycosis fungoides (poikiloderma vasculare
atrophicans),
• Borrelia infection (acrodermatitis chronica atrophicans),
• chronic radiation dermatitis,
• graft-versus-host disease, hydroxyurea,
• and dyskeratosis congenita.

19. • Panniculitis mencerminkan penyakit aktif pada DM, biasanya
mempengaruhi bokong, batang tubuh, dan ekstremitas
proksimal dapat berkembang menjadi kalsinosis atau
lipoatrofi.
• Histopatologi menunjukkan panniculitis lobular tetapi
mungkin memiliki fitur lupus panniculitis dengan perubahan
lipomembran atau dengan penebalan septum seperti pada
morfea dalam.

20. • Alopecia in DM is most commonly nonscarring and diffuse,
although patchy involvement, rarely with scarring, can also
be seen.
• Alopecia can be caused by DM disease, coexisting disorders,
medications, or telogen effluvium.
• Patients with anti-MDA5 antibodies have a higher risk of
alopecia, which commonly is severe and occurs early in the
disease.

22. EXTRACUTANEOUS FINDINGS
• PULMONARY INVOLVEMENT
• Other pulmonary manifestations in DM include aspiration pneumonia; drug-induced pneumonitis; and, rarely,
pulmonary hypertension
• MUSCLE INVOLVEMENT
• Myositis in DM typically presents as symmetrical proximal muscle weakness.
• Patients may also complain of myalgias, which can occur even in the absence of frank clinical weakness.
• INTERNAL MALIGNANCY
• Common types of solid tumors include breast, lung, ovarian, prostate, colorectal, gastric, and pancreatic,
nasopharyngeal cancer being more common in Southeast Asians.
• CARDIOVASCULAR INVOLVEMENT
• ST-T segment changes, left ventricular hypertrophy, Myocarditis
• GASTROINTESTINAL INVOLVEMENT
• result from a vasculopathy affecting the bowel wall.
• JOINT INVOLVEMENT
• Arthralgias are common in DM, reported in 30% to 40% of patients

23. ETIOLOGY AND PATHOGENESIS
• DM is primarily an immune-mediated disorder with molecular and
histologic evidence supporting a role for both innate and adaptive
immunity
• skin biopsies show infiltration of CD3+ T cells, plasmacytoid dendritic
cells, and macrophages as well as B cells (especially in muscle)
• Parenchymal cell injury is manifested in the skin by interface
dermatitis with keratinocyte injury and in muscle by atrophy,
degeneration, and regeneration of muscle fibers, typically in a
perifascicular distribution.

24. DIAGNOSIS / HELPFUL SKIN FINDINGS
• Features of the skin examination that we find particularly sensitive
are
• microscopic periungual telangiectasias,
• lateral digit hyperkeratosis, and scalp erythema and dysesthesia.
• Some elements that are more specific are the “red-on-white” patches,
• the ovoid palatal patch,
• grossly visible periungual telangiectasias,
• and Gottron papules.

25. • Muscle involvement by history (including dysphagia,
dysphonia, and myalgia) and weakness on examination will
serve to increase suspicion for myositis but are not
confirmatory.
• Elevation of muscle enzymes,
• Electromyography or MRI can be used in situations when the
clinical suspicion is still high for myopathy but muscle enzymes are
normal.
• Finally, a muscle biopsy can be performed in cases in which a
cause for muscle symptoms is still not clear.

26. AUTOANTIBODIES
• Myositis-specific autoantibodies associated with DM are evolving as key tools in
assisting in establishing the diagnosis.
• These MSAs include TIF1-γ, NXP2, MDA5, small ubiquitin-like modifier activating
enzyme (SAE), Mi-2, Jo-1, and the other antisynthetase antibodies (PL7, PL-12, EJ,
OJ, SRP)
• ANA testing, if performed, should be done using method of direct
immunofluorescence.
• ANA test results can be negative in DM 50% of the time but are usually positive in
systemic lupus erythematosus, and thus a negative test result can help point
away from systemic lupus if that is in the differential diagnosis.
• serum ferritin is often highly elevated (>500 mg/dL) in anti-MDA5 patients with
DM111 and may be helpful in diagnosis anti-MDA5 disease when serologic testing
is unavailable or of inadequate sensitivity.

27. IMAGING
• MRI provides a detailed view of the muscle anatomy, allowing for
localization and discrimination of the type of pathologic process (eg,
edema, inflammation, fibrosis, calcifications, or atrophy).
• MRI can be useful to differentiate weakness caused by damage or
steroid-myopathy versus active myositis when muscle enzymes and
electromyographic studies are inconclusive.

28. Skin / muscle biopsies
from affected area in DM
SKIN
• a cell-poor interface dermatitis,
• increased dermal mucin,
• perivascular lymphocytic infiltrate,
• and vascular ectasia
MUSCLE
• perifascicular atrophy,
• degenerating and regenerating myofibers,
• membrane attack complex deposition in the endomysial
capillary walls,
• endothelial cell swelling, and capillary necrosis.
• The inflammatory cell infiltrate consists of CD4+ T cells,
plasmacytoid dendritic cells secreting IFN-α,B
lymphocytes, macrophages, and plasma cells.

30. • Pengobatan DM terlebih dahulu memerlukan penilaian organ yang
berpotensi terkena, yaitu kulit, otot, dan paru-paru.
• Harus ditekankan bahwa ILD dan kanker terkait adalah penyebab utama
kematian terkait penyakit dan diprioritaskan selama pemilihan
pengobatan.
• Skrining untuk keganasan sangat penting karena pengobatan kanker yang
terkait dengan DM dapat mengakibatkan penurunan keparahan penyakit
atau, kadang-kadang, remisi penyakit.
• DM kulit sering memiliki respon pengobatan berbeda dengan penyakit
otot, dengan penyakit kulit lebih sulit sembuh dan berlanjut bertahun-
tahun setelah penyakit otot dalam remisi.
• cancer screening studies (colonoscopy, mammogram, prostate
examination) and relevant screening blood work (complete blood count,
renal and liver function tests) as well as a urinalysis are indicated.

31. TOPICAL THERAPY
• Meskipun fotoproteksi merupakan langkah kunci pertama dalam
manajemen, hingga 60% pasien dengan DM sebenarnya fotosensitif
minimal, dan hanya 20% dapat melaporkan eksaserbasi penyakit
setelah paparan sinar UV.
• Kortikosteroid topikal mungkin memiliki efek paliatif pada penyakit
kulit DM dengan mengurangi eritema, skala, dan pruritus dan
memainkan peran tambahan untuk agen sistemik.
• Inhibitor kalsineurin topikal seperti salep tacrolimus 0,1% atau krim
pimecrolimus 1% mungkin memiliki kemanjuran sederhana dalam
beberapa kasus DM kulit.