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What is Scleroderma?

Sclerodermia is a chronic connective tissue disease, or systemic sclerosis, commonly known as one autoimmune rheumatic disease.

The word “scleroderma” is derived from the Greek word “sclero” which means hard and “derma” which means skin. Skin hardening is one of the disease’s most visible manifestations.

The condition was called “progressive systemic sclerosis,” but the use of that term was discouraged because scleroderma was found to be not necessarily progressive. The disease depends on the patient.

Symptoms Of Scleroderma

The symptoms of Scleroderma are given below:

i) Raynaud ‘s phenomenon (presented as a symptom in 30% of affected individuals, occurs in 95% of the people affected sometime during illness), healed pitting fingertip ulcers, skin and mucosal telangiectasis, palpitations, irregular heart rate and disappearance due to abnormalities of conduct, hypertension and congestive insufficiency of the heart.

ii) Digestion: gastroesophageal reflux disorder, blowing, indigestion, loss of appetite, alternating diarrhoea, sicca syndrome and its symptoms, teeth loosening and hoarseness (because of reflux of acids).

iii) Pulmonary: progressive deterioration in shortness of breath , chest pain, dry persistent toxin due to interstitial lung disorder (pulmonary artery hypertension).

iv) Musculoskeletal: joint, muscle aches, loss of joint movement, syndrome in the carpal tunnel, and weakness in muscle.

v) erectile dysfunction, dyspareunia, problems with the kidney, or kidney failure.

vi) Similarly, facial pain, headache, stroke, fatigue, calcination, and weight loss. Such as a result of trigeminal neuralgia.

Causes Of Scleroderma

Genetic and environmental factors are responsible for sclerodermia. In certain cases, mutations in HLA genes appear to be a crucial factor in pathogenesis, but not all, and in a limited proportion of people affected, the exposure to silica, aromatic, chlorinated solvency, ketones, trichloroethylene, sweat fumes and white spirits is also a major contributor.

Treatment Of Scleroderma

The following measures are the ways to treat Scleroderma:

i) Phenomenon of Raynaud with vasodilators such as blockers of calcium channels, alpha blockers, serotonin receptor antagonists, inhibitors of angiotensin II, statins, local nitrates, or iloprost.

ii) Phosphodiesterase 5 (e.g. sildenafil) or iloprost digital ulcers.

iii) Prevention of new bosentan digital ulcers.

iv) Tetracycline Antibiotics like tetracycline Malnutrition, secondary to intestinal flora,

v) Cyclophosphamide, azathioprine interstitial lung disease with or without corticosteroids.

vi) Endotheline-receptor antagonist, 5-inhibitor phosphodiesterase and prostanoids pulmonary artery hypertension.

vii) Antacid or prokinetic gastrooesophageal reflux disease.

viii) Angiotensin converting angiotensin inhibitors and angiotensin II receptor adversaries kidney crises.

Classification Of Scleroderma

It is classified into 2 types and they have their sub types:

1) Localised scleroderma
i) Localised morphea
ii) Morphea-lichen sclerosus et atrophicus overlap
iii) Generalised morphea
iv) Atrophoderma of Pasini and Pierini
v) Pansclerotic morphea
vi) Morphea profunda
vii) Linear scleroderma
2) Systemic scleroderma
i) CREST syndrome
ii) Progressive systemic sclerosis