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.

41. Early and late

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