A 52 year old female came to OPD with dysphagia, hea burn and pain in joints of her fingers. She mentions that her fingers and toes turns blue on exposure to cold. Physical examination reveals thickened skin of her fingers and tender, painful small lumps under skin of flexor surface of hand, some of which drain white chalky substance. what is the clinical diagnosis?
The most likely diagnosis for the above given clinical scenario is CREST Syndrome. CREST SYNDROME * It is a multisystem connective tissue disorder, which is a limited cutaneous form of Systemic sclerosis. * Cardinal features: C- Calcinosis : It is the pathologic calcification of soft tissues, which can present as tender and painful small lumps most commonly on flexor aspect of hands or extensor surface of forearm. They can ulcerate and drain a white chalky substance and can be secondarily infected. R- Raynaud phenomenon : episodes of pallor, cyanosis, and/or rubor on the hands bilaterally in response to cold or emotional stress. E- Esophageal dysmotility : progressive atrophy and collagenous fibrous replacement of the muscularis propria, leads to hypomotility of esophagus and dysphagia. S- Sclerodactyly : It is thickening of the skin of the digits of the hands and feet. Three phases of skin changes are seen in scleroderma: the edematous phase, indurative phase, and atrophic phase. T- Telengiectasia :These are lesions formed by collections of dilated blood vessels and may be highlighted by dermoscopy. *The most characteristic antibody in CREST syndrome is anti-centromere antibody (against centromeric proteins).
Systemic lupus erythematosus
Sjogren Syndrome
CREST syndrome
Mikulicz syndrome
Other Autoimmune Disorders