Unusual presentation of progressive systemic sclerosis

A female 38 years old, housewife, presented to the Department of Dermatology and Venereology, Mymensingh Medical College Hospital (MMCH) on 08.04.07 with the complaints of i) pain and reduced movement of hand, knee, shoulder and neck joints for 1 year and 9 months ii) tightness of skin over face, neck, limbs and trunk for 1 year and 6 months iii) patchy depigmentation over same areas for 1 year and 3 months iv) deformity of hands with flexion contractures for 6 months and v) dysphagia to solid food for 3 months. She had no complaints of Raynaud's phenomenon. On general examination, she was ill looking, anemic and nutritionally poor. Examination of integumentary system showed smooth, shiny, thick, hard and hidebound skin with pigmentary alteration of 'salt and pepper' appearance over fingers, hands, limbs, face, neck and trunk. Hands appear claw like but more on the right side than the left and there were no other obvious changes suggestive of digital ischaemia (atrophy, ulceration, scarring, gangrene etc). Face has got suggestive features of scleroderma. Examination of the respiratory system showed restriction of chest movement and reduced expansibility of chest wall. No other abnormality was found on examination of other systems. Laboratory investigations showed histopathology typical of scleroderma. X-ray of hands and feet showed suggestive changes, lung function test-showed restrictive lung disease. Barium swallow x-ray of esophagus in supine position showed mild dilation of lower oesophagus. But serology was non-reactive (negative ANA, Negative RA test, Negative VDRL). So, she was diagnosed as a case of progressive systemic sclerosis (PSS) with some atypicality.