Telangiectasias with tight skin
A 49-year-old woman presented to her family physician (FP) with skin changes on her face and hands and gastroesophageal reflux disease. The FP examined the patient’s face, which showed multiple telangiectasias and tight skin over her nose and around her mouth (top image). Her hands exhibited tight skin over the fingers with some deformities due to the tightening of the skin (bottom image). When he examined an individual finger, the FP noticed pitting of the skin and loss of the soft tissue of the distal phalanx.
What’s your diagnosis?
The FP suspected that the patient had CREST (calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias) syndrome also known as limited cutaneous systemic sclerosis (LcSSc). He examined her arms and found that she had firm nodules around the elbows consistent with calcinosis. Further history revealed Raynaud's phenomenon. This clinched the diagnosis of CREST syndrome. The FP ordered blood tests, a chest x-ray (CXR), and pulmonary function tests to determine if there was pulmonary involvement and to learn more about possible systemic effects of her disease.
Systemic sclerosis (scleroderma) is characterized by skin induration and thickening accompanied by variable tissue fibrosis and inflammatory infiltration in numerous visceral organs. Systemic sclerosis can be diffuse or limited to the skin and adjacent tissues (LcSSc). Patients with LcSSc usually have skin sclerosis that is restricted to the hands and, to a lesser extent, the face and neck.
,The patient’s antinuclear antibody test was positive with a speckled nucleolar staining pattern, which is a common finding in systemic sclerosis. Her CXR showed evidence of interstitial lung disease with a ground glass pattern. Her pulmonary function test showed a diminished diffusion capacity. Pulmonary disease is seen in all types of systemic sclerosis and not diagnostic for one type. The patient was referred to Rheumatology for further diagnosis and management.
Photos and text for Photo Rounds Friday courtesy of Richard P. Usatine, MD. This case was adapted from: Mayeaux EJ, Usatine R. Scleroderma and morphea. In: Usatine R, Smith M, Mayeaux EJ, et al. eds. Color Atlas and Synopsis of Family Medicine. 3rd ed. New York, NY: McGraw-Hill; 2019:1204-1212.
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