Saturday, October 27, 2007

Dermatomyositis

Dermatomyositis Dermatomyositis is a chronic inflammatory disorder of skin, subcutaneous fat as well as skeletal muscle. It is characterized by distinctive skin rash and edema which induce muscle weakness, it may accompanied by athralgia (pain in joint). The cause of dermatomyositis is unknown; recently it is said to be related to anti-immune disease, abnormal reaction towards viral infection and tumor and etc.Clinical manifestations: In early stage, the signs are general weakness, headache, athralgia, slight muscle weakness and others; main pathological changes is at the skin and muscle. Polymyositis is inflammatory myopathy without involving the skin. Mostly seen in middle-ages women.(1) skin symptoms: it begins by edematous rashes especially at the eyelids with purple discoloration, while the cheek may have diffuse chronic dusky patches. Besides, there is dilatation of capillaries, similar to SLE; body dehydration, skin dryness with scalling erythematous eruption and minor skin atrophy, capillaries dilatation and skin discoloration. The skin of posterior surface of fingers joint yields significant edematous rashes, pigmentation, sometimes would appear purple papules. Rashes around nails. 30% of patients has Reynaud phenomenon as early symptoms. 20% of juvenile dermamyositis has deposition of calcium in the muscles or under the skin.(2) muscular symptoms: in acute period, there is myositis (inflammaion of muscle), muscular weakness, swollen muscle, origin pain or compression pain. Generally, proximal muscle is first affected, appearance of movement disturbances such as raising hand and leg, squatting, swallowing, speaking and so on. There will be different symptoms according to different muscular damages. The chronic dermatomyositis has muscular atrophy or sclerosis and leads to complete loss of physical ability. Other complications are such as periodical pneumonitis, myocarditis, athritis, glomerulonephritis and widespread of vasculitis. According to several studies, about 5-40% of patients have the risk of cancer, often combined with lung tumor, digestive tract tumor, stomach tumor, lymphosarcoma and etc. Cancer can happens before, during or after dermatomyositis. Juvenile dermatomyositis seldoms accompanied by cancer.Laboratory analysis:1. Quantity of creatine in 24 hours urine is increased, may more than 1000 milligram (norm: 0-200 milligram).2. Blood serum enzymes: significant increase of ASAT (aspartate aminotransferase, previously known as GOT- glutamate oxaloacetate transaminase), LDH (lactate dehydrogenase), CPK (creatine-phosphate-kinase), CK (creatinine kinase), aldolase; CPK and aldolase often follow the pathological changes of muscle.3. Immunological analysis: increase of immunoglobulins, ESR (erythrocyte sedimentation rate), RA positive.4. Electromyography (EMG): myopathic and atrophic EMG. During low voltage, the oscillation amplitude reduces, the unit reduces, appears of multihomogeneous unit, unit s duration is reduced and etc. 5. Pathology: Skin: Epidermis basal cell undergoes liquefication, basal membrane s subcutaneous shows positive in PAS dye. The upper part of dermis has edema, sedimentation of amyloid protein, swelling of collagen fiber and vasodilatation. Muscle: (1) allergic reaction: mild case: The lines of muscle fiber vanish, coagulation of muscular fluid, transparent changes, proliferation of cells. Severe case: The breaking of muscle fiber, abnormal reaction of macrophages. (2)Inflammation: The infiltration of inflammatory germ cells, edematous muscular fibers, vasodilatation. In late period may appear sclerosis of muscular fibers, atrophy, substitute by connective tissue after its degeneration.Diagnosis:(1) muscular symptoms:1. acute, subacute or chronic process.2. sometimes accompanied by myalgia (pain in muscle).3. decrease ability of muscles of extremities (especially proxiaml muscle), face, neck, pharynx and larynx muscle and etc or muscular atrophy but it must be differentiated from other connective tissue disease which is along with myopathies.(2) skin symptoms: symmetrical distribution on face, chest, limbs (extensor surface), especially the joint, back and etc. 1. purplish-red edematous patches (particularly eyelid)2. blood capillary dilatation, pigmentation, atrophy.3. Raynaud symptom.4. joints pains.Treatment:The western medicine uses 1.cortical hormone 2.immunosuppresors 3.vitamins 4.muscular medicines 5.proteins assimilated medicines 6 .supportive therapy.Traditional Chinese medicine (TCM) treatment. Heat type: clear and nutritive to blood and disintoxicating; cold type: eliminate cold and invigorates blood; weak type: adjustment of yin and yang , invigorates qi (vital energy) and blood.[Clinical treatment result] Treated 513 cases, after 1 month of medicine introduction, 496 cases significantly effective, effective17 cases.

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