A 35 year old lady complains of dysphagia, Raynaud's phenomenon and her hands show the following appearance. Investigations show positive ANA. The likely diagnosis is
In the image, extensive subcutaneous fibrosis has viually immobilized the fingers, creating a clawlike flexion deformity.Systemic sclerosis is characterized by: (1) Chronic inflammation thought to be the result of autoimmunity, (2) Widespread damage to small blood vessels, and (3) Progressive interstitial and perivascular fibrosis in the skin and multiple organs.Skin thickening, Raynaud phenomenon, manifested as episodic vasoconstriction of the aeries and aerioles of the extremities is seen in viually all patients and precedes other symptoms in 70% of cases.Dysphagia attributable to oesophagal fibrosis and its resultant hypomotility is present in more than 50% of patients.Viually all patients have ANAs, one of these is directed against DNA topoisomerase I (anti-Scl 70), another, an anticentromere antibody (CREST syndrome)Ref: Robbins Pathology; 9th edition; Page no: 230
Systemic lupus erythematosus
Systemic sclerosis
Mixed connective tissue disorder
Rheumatoid ahritis
General pathology