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Chapter-22 Systemic Sclerosis

BOOK TITLE: Essential of Dermatology, Venereology and Leprosy

Author
1. Chattopadhyay SP
ISBN
9788180610172
DOI
10.5005/jp/books/11611_22
Edition
1/e
Publishing Year
2003
Pages
4
Author Affiliations
1. Armed Forces Medical College Pune, Vivekananda Institute of Medical Sciences, Ramakrishna Mission Seva Pratishthan; Calcutta Medical and Research Institute, Kolkata, West Bengal, India
Chapter keywords
Chronic connective tissue disorder predisposition—precipitation factor, Clinical picture diagnosis, Skin biopsy confirms, No specific treatment vasodilators, Nifedipine, Prostaglandin F1, Prostacyclin, D-penicillamine.

Abstract

Systemic sclerosis: Chronic connective tissue disorder. Raynaud’s phenomenon, atrophic and sclerotic change associate with widespread vasculitis in skin and internal organs. 1. Genetic predisposition: HLA B8. 2. Autoimmune factor. 3. Precipitating factors: Silica exposure, viral infection, endocrinal abnormalities. Clinical features: Earliest one is Raynaud’s phenomenon associated with swelling hands, joint swellings, fingertip ulceration. Patient may present with leg ulcers or weight loss, dyspnoea, radiological change in lungs confusing with tuberculous infection. Face: (i) Forehead smooth and shiny. (ii) Nose—small and pinhead. (iii) Mouth—opening constricted. (iv) Lower eyelids cannot be depressed. (v) Telangiectasia frequently seen. Chest: Pigmentation Hyperpigmentation is seen in 50 percent cases hypo-pigmentation may be seen over fingers and face. Systemic involvement: GIT Involvement is common. Tongue–Macroglossia: Pulmonary involvement: Pulmonary function test abnormality in 70 percent. Cardiac involvement ECG abnormality in 50 percent. Renal involvement. It is common proteinuria is earliest and most common; in 40 percent cases hypertension occurs. Investigations: Skin biopsy is the only confirmatory investigation, rest are contributory. Antinuclear antibody: It is positive in 75 percent. Anti-DNA antibody: It is absent. Treatment: Hence management of symptoms and patient as whole, corticosteroids in low does only give feeling of well-being and do not alter the course of disease: 1. Prevention from truma (fingers) and 2. Smoking is discouraged. Drugs: Vasodilators, nifedipine—10–20 mg TDS, and new drugs—prostaglandin F1 and prostacyclin.

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