Focal linear elastosis in a patient with joint hypermobility syndrome

Abstract

Focal linear elastosis (FLE) is a benign skin findingcharacterized by hypertrophic linear plaques withabnormal elastic fibers on histology. We present aunique case in which focal linear elastosis occurredin the setting of joint hypermobility syndrome(JHS). Our patient, a 20-year-old man with a medicalhistory significant for symptoms consistent with JHS,had been followed by the rheumatology clinic formany months. He was referred to the dermatologydepartment for further evaluation of asymptomaticlongitudinal bands on his back that had been presentfor many years. He denied trauma but endorsed ahistory of 'stretch marks.' On examination there werenumerous horizontally oriented, firm, linear, yellowto flesh colored bands, all non-tender to palpation.Punch biopsies were performed of involved anduninvolved skin. Histopathology of normal skinrevealed no significant abnormalities whereasinvolved skin demonstrated broadened collagenbundles in the deep dermis. The elastic fiber stain,Verhoeff-Van Gieson, revealed a gross increase in thenumber of elastic fibers, fragmented fibers, fiberswith "paintbrush" or widened-ends, fibers of varyingthickness, and clumped fibers. This combination ofhistopathologic and clinical features was consistent with FLE.