Which of the following conditions is NOT commonly associated with this finding? A) Chronic liver disease B) Cutaneous collagenous vasculopathy C) Hemophilia A D) CREST syndrome E) Rosacea

SYSTEMIC SCLEROSIS. Systemic sclerosis (ssc) /Scleroderma is a rare chronic disease of unknown etiology characterised by diffuse fibrosis. Degenerative changes and Vascular abnormalities in the skin ,joints and internal organs. COMMON SYMPTOMS. Raynaud's phenomenon. Polyarthritis. Dysphagia. Heartburn. Swelling of fingers. Skin tightening and contracture of fingers. Lung ,kidney and heart involved. ETIOLOGY. Heridity and immunological mechanisms play a role in etiology. Scleroderma like syndrome can also result from exposure to vinyl chloride ,bleomycin ,pentazocine , epoxy ,aromatic hydrocarbons ,contaminated rapeseed oil or L -tryptophan. PATHOPHYSIOLOGY Pathophysiology involves vascular damage and activation of fibroblasts. Changes in the skin. *skin develops compact collagen fibers in the reticular dermis, *epidermal thinning. *loss of rete pegs. *atrophy of dermal appendages. *accumulation of T cells. *extensive fibrosis of the subcutaneous and dermal layers. *capillary loops are dilated and microvascular loops are lost kn the nail bed. *chronic inflammation and fibrosis of synovial membranes. Changes in the GIT. *esophageal motility is impaired. *lower esophageal sphincter becomes incompetent. *secondary strictures develop. *intestinal muscularis mucosa degenerates. leading to pseudo diverticula in thecolon and ileum. Changes in respiratory tract. *interstitial and peri bronchiolar fibrosis. *intimal hyperplasia of small pulmonary atteries. *pulmonary hypertension. Changes in heart. *diffuse myocardial fibrosis. *cardiac conduction abnormalities. There are Two types of systemic sclerosis. 1) LIMITED CUTANEOUS SYSTEMIC SCLEROSIS / LIMITED SCLERODERMA. Thid is also known as CREST SYNDROME Calcinosis. Raynaud's phenomenon. Esophageal dysmotility. Sclerodactyly. Telangiectasia. 2)DIFFUSE CUTANEOUS SYSTEMIC SCLEROSIS. OR DIFFUSE SCLERODERMA. SYMPTOMS AND SOGNS. The most common symptoms are Raynaud's phenomenon and insidious swelling of distal exyremities. SKIN AND NAIL MANIFESTATIONS *Symmetric swelling of the skin which progresses to induration. *Sclerodactyly. *salt and pepper appearance of skin. *subcutaneous calcifications. *digital ulcers. *skin becomes shiny ,taut ,hypopigmented or hyperpigment JOINT MANIFESTATIONS. *Polyarthralgia. *flexion contractures . GI MANIFESTATIONS. *Esophageal dysfunction. *Retro sternal Dysphagia. *diverticula in the colon. CARDIO PULMONARY MANIFESTATIONS. *Lung fibrosis. Acute alveolotis. Pericarditis with pleural effusion. *Cardiac arrhythmias. RENAL MANIFESTATIONS. Renal disease. DIAGNOSIS *Clinical evaluation Usually ANTI NUCLEAR ANTIBODIES (ANA ), Scl.-70 (topoisomerase. Anti centromere antibodies. PROGNOSIS. Slow progression of diseasr. 10 yr survival rate is 65 % TREATMENT. Treatment is directed at symptoms and dysfunctional organs. KEY POINTS. Pathologic changes include skin and joint changes. Raynaud's phenomenon. Esophageal dysfunction. Involvement of lung,heart and kidneys. Consider the diagnosis in Raynaud's phenomenon. Typical musculoskeletal or skin manifestations. Unexplained Dysphagia. Malabsorption. Pulmonary fibrosis. Pulmonary hypertension. Cardiomyopathies. Conduction disturbances. Test for ANA ,Scl-70 (topoisomerase), and anti centromere antibodies. Because there is no clear disease modifying therapy ,treatment is directed at the involved organs.

45 years old lady was presented with history of dry cough, GERD symptoms, significant weight loss, her hands as you see in the attached photo, so what's your thoughts about the exact diagnosis, investigations and management ?

Laboratory diagnosis shows anticentromere antibodies and below is the X-ray findings. What is the probable diagnosis and treatment?

43 year old man came with depigmentation in the ears, diagnosed as vitiligo. On additional examination as shown in the photos... A clinical diagnosis was made. Patient had difficulty in swallowing. 1. Identify what is being demonstrated. 2. Identify the condition 3. Which blood test will help confirm the diagnosis.