Dermatology - Scleroderma
Inflammatory, vascular, and sclerotic alterations of the skin and several internal organs, including the heart, lungs, and gastrointestinal tract, are the hallmarks of scleroderma, a multisystem condition. 60% of instances with scleroderma are restricted, and 40% are diffuse; the latter has a high rate of morbidity and death. It strikes between the ages of 30 and 50, with women four times more likely to be impacted than males. There is always secondary Raynaud phenomenon and skin sclerosis. Additional symptoms include heartburn, migratory polyarthritis, arthralgia, constipation, diarrhea, bloating in the abdomen, malabsorption, weight loss, exertional dyspnea, and dry cough. The Raynaud phenomenon occurs months or years before sclerosis. Next, there is painful ulcerations at the fingertips accompanied by nonpitting edema of the hands and feet that heals with pitted scars. Later on, sclerodactyly develops, characterized by tapering fingers and skin that is glossy, waxy, and rigid, tightly bound to prevent folding or wrinkles. Synovial flexion contractures cause leathery crepitation over those joints. Nails with shorter distal phalanges become claw-like due to periungual telangiectasia. Distal phalanges are lost as a consequence of resorption of bone and ulcerations. On the distal extremities, there is total hair loss, thinning, and anhidrosis associated with the loss of sweat glands. Periorbital edema is the first symptom, and more edema and fibrosis cause mask-like facies, radial perioral furrowing, thinning of the lips, beak-like pointed nose, and loss of normal facial lines. There is diffuse hyperpigmentation and telangiectasia. Diffuse scleroderma is characterized by early involvement of the chest and proximal upper and lower extremities, as well as tense, stiff, and waxy-looking skin that is impossible to fold and limited chest wall movement, which impairs breathing. Clinical findings are used to make the diagnosis, which is then verified by dermatopathology. Mixed connective tissue disease, eosinophilic fasciitis, scleromyxedema, morphea, porphyria cutanea tarda, adverse drug responses (bleomycin, pentazocine), polyvinyl chloride exposure, lichen sclerosus et atrophicus, and systemic fibrosis are among the conditions included in the differential. Early in the disease, systemic glucocorticoids may be helpful for brief periods of time. Immunosuppressive medications such as mycophenolate mofetil, cyclosporine, methotrexate, and cyclophosphamide have improved skin, but their effects on systemic involvement have been somewhat restricted.
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