Chest
Selected ReportsSevere Extrapulmonary Thoracic Restriction Caused by Morphea, a Form of Localized Scleroderma
Section snippets
Case Report
A 64-year-old man was evaluated because of progressive dyspnea that was thought to he secondary to parenchymal lung involvement by scleroderma. He had been treated with oral prednisone and azathioprine for the last year. Ten years prior to our evaluation, the patient complained of skin tightness and presented with skin hyperpigmentation and thickening localized to his shoulders and thoracoabdominal region. A punch biopsy specimen of the skin revealed thick bundles of collagen between the
Discussion
Although the histologic pattern of cutaneous sclerosis in morphea is similar to scleroderma, morphea is rarely associated with visceral involvement, acral areas tend to be spared, and the incidence of positive antinuclear antibodies is low.1 In addition, spontaneous resolution or improvement of cutaneous sclerosis is not uncommon in morphea.1 We describe a patient with generalized morphea and severe extrapulmonary thoracic restriction in whom the predominant mechanism for restriction appears to
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Cited by (5)
Autoantibodies
2021, Encyclopedia of Respiratory Medicine, Second EditionLung Disease Secondary to Morphea en Coup de Sabre, a Form of Localized Scleroderma
2009, Archivos de BronconeumologiaClinical profile, long-Term follow-up and outcome of juvenile systemic scleroderma: 25 years of clinical experience from North-West India
2021, Clinical and Experimental RheumatologyScleroderma lung disease
2013, European Respiratory ReviewMorphea, a review
2007, Dermatologia Cosmetica, Medica y Quirurgica
Drs. Aguayo and Roman are recipients of Minority Faculty Development Awards from the Robert Wood Johnson Foundation. This work was also supported by grants from the American Lung Association and the Research Advisory Group from the Department of Veterans Affairs.