A 51-year-old post-menopausal African American female presents to the clinic complaining of excessive pitting of her fingertips, which affects her daily life as a piano player. Her past medical history includes burning chest pain and dysphasia. Her vital signs are normal. On physical examination, the patient has skin thickening on her face and hands, facial telangiectasia and hardening and tapering of her fingertips. Serologic studies are positive for antinuclear antibodies in a centromere pattern. She does not use tobacco, drink alcohol, or use illicit drugs. She is a mother of two healthy children and her family history is unremarkable.
The correct answer is: E. Tricolor change of finger and toes
The patient signs and symptoms of pitting fingertips (i.e. calcinosis cutis), burning chest pain, dysphasia, telangiectasia and hard thickening of fingers (i.e. sclerodactyly), make the diagnosis of CREST syndrome the most likely one (Answer E). CREST syndrome is a localized variant of systemic sclerosis characterized by the presence of Calcinosis, Raynaud's phenomenon (i.e. three-color change of fingers and toes), esophageal dysmotility, sclerodactyly and telangiectasias.1
Furthermore, serologic studies usually are positive for anti-centromere antibodies. Raynaud's syndrome presents with the characteristic three-color change featuring pallor (ischemic phase), cyanosis (deoxygenation phase), and erythema (reperfusion phase) induced by cold or stress. Although the underlying pathophysiological mechanism is unclear, alterations in activity of the peripheral adrenoceptor have been implicated.
There are two ways that Raynaud's syndrome could be idiopathic (i.e. primary) or secondary, being commonly associated with connective tissue disorders (e.g. Sjogren's syndrome, systemic lupus erythematosus, scleroderma etc.).2 The estimated prevalence in the general population ranges from 3% to 5%, with a higher prevalence in women than in men.
Papules and plaques with silvery scaling describes the characteristic findings in psoriasis. Although the patient presents with some dermatological manifestations (sclerodactyly and calcinosis cutis), no lesions indicating psoriasis are revealed by the clinical examination. (Answer A)
Joint fusion of sacroiliac joints is the characteristic feature of ankylosing spondylitis. Ankylosing spondylitis is a seronegative spondyloarthritis, most commonly found in males. There is no known association between CREST syndrome and ankylosing spondylitis. (Answer B)
Facial malar rash might be a feature of systemic lupus erythematosus or dermatomyositis. However, both of those diseases cannot be explained by the rest findings of clinical examination. Furthermore, serological testing would be positive for anti-Sm or anti-dsDNA (SLE) and anti-Mi-2(dermatomyositis) antibodies. (Answer C).
Dry cough and bilateral mediastinal adenopathy are characteristic symptoms of sarcoidosis. Although the patient is an African American and female, the rest of the findings cannot be explained by this diagnosis. (Answer D)
Educational objective:
Raynaud's phenomenon describes the decreased blood flow to skin due to arteriolar vasospasm in response to cold or stress. This disorder is called Raynaud disease when it is idiopathic and Raynaud's syndrome when it is secondary to a disease process such as CREST syndrome.1 The proper treatment usually includes the usage of calcium channel blockers.2
References
- Williams, E. CREST syndrome, a case report. J Am Acad Dermatol 2017;76:AB85
- Valdovinos ST, Landry GJ. Raynaud syndrome. Tech Vasc Interv Radiol 2014;17:241-6.
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